Materiały źródłowe

• #1 Calciphylaxis (calcific uremic arteriolopathy) – UpToDate

  https://www.uptodate.com/contents/calciphylaxis-calcific-uremic-arteriolopathy

  Calciphylaxis is a rare and serious disorder that presents with skin ischemia and necrosis and is characterized histologically by calcification of arterioles and capillaries in the dermis and subcutaneous adipose tissue. […] This topic reviews the pathogenesis, diagnosis, and treatment of calciphylaxis in ESKD (calcific uremic arteriolopathy) and non-ESKD patients. […] There is no approved treatment for calciphylaxis. […] Calciphylaxis most commonly occurs in patients who have end-stage kidney disease (ESKD) and are on dialysis but may also occur in kidney transplant recipients and in non-ESKD patients. […] When to suspect calciphylaxis […] Confirming the diagnosis.

• #2 Calciphylaxis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/calciphylaxis?lang=us

  Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare condition that manifests as subcutaneous vascular calcification and cutaneous necrosis (small blood vessels of the fat tissue and the skin). Some authors describe it as a syndrome of vascular calcification, thrombosis and skin necrosis. […] The exact pathogenesis of calciphylaxis is unclear. Medial calcification and intimal fibrosis of the cutaneous arterioles combined with thrombotic occlusion leading to ischemic skin necrosis is seen in calciphylaxis. […] More than 50 percent of patients die (most commonly from sepsis) within one year of being diagnosed.

• #3 Calciphylaxis in patients with chronic kidney disease: A disease which is still bewildering and potentially fatal | Nefrología

  https://www.revistanefrologia.com/en-calciphylaxis-in-patients-with-chronic-articulo-resumen-S2013251418301068

  Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare syndrome that typically causes skin necrosis and usually affects dialysis patients. […] From a clinical point of view, calciphylaxis may progress from painful purpura to extensive areas of skin necrosis that can potentially lead to superinfection and the death of the patient due to sepsis. […] Calciphylaxis, also called calcific uremic arteriolopathy (CUA), is a clinical syndrome characterized by necrotic ulceration of the skin due to arteriolar calcification of the media plus fibrosis of the intima and subsequent cutaneous ischemia due to thrombosis of the arteriole. […] The diagnosis is mainly clinical, although the role of skin biopsy is very important, especially in doubtful cases. […] The skin biopsy would confirm the diagnosis of calciphylaxis.

• #4 Calciphylaxis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/calciphylaxis/symptoms-causes/syc-20370559

  Calciphylaxis treatments include various medicines, procedures and surgery. Treatment can help prevent blood clots and infections, reduce calcium buildups, heal sores, and ease pain. […] The exact cause of calciphylaxis isn’t known. The disease involves the buildup of calcium in the smallest parts of the arteries in fat tissues and skin. […] Finding and treating any infections early is key to preventing serious complications. […] There isn’t a clear way to prevent calciphylaxis. But if you are on dialysis or have low kidney function due to advanced chronic kidney disease, it’s important to keep blood levels of calcium and phosphorus under control.

• #5

  https://www.kidney.org.uk/calciphylaxis-information

  Calciphylaxis is a serious condition that can occur in people chronic with kidney disease; it is most commonly seen in people receiving dialysis but remains very rare with approximately one case diagnosed in every 600 people, having dialysis, per year. […] There is no diagnostic test for calciphylaxis. Diagnosis is made by clinical examination. Sometimes a skin biopsy can be taken (a small sample of skin is removed and tested) to check for evidence of calcification but this isnt always necessary. […] There are no specific treatments and therefore no specialist hospital that treats calciphylaxis. There are healthcare professionals in the UK that have an interest in calciphylaxis and your kidney team can contact them for advice (through the calciphylaxis rare disease group).

• #6 Diagnosis and treatment of calciphylaxis in patients with chronic kidney disease – Brazilian Journal of Nephrology (BJN)

  https://www.bjnephrology.org/en/article/diagnosis-and-treatment-of-calciphylaxis-in-patients-with-chronic-kidney-disease/

  Diagnosis and treatment of calciphylaxis in patients with chronic kidney disease […] Calciphylaxis is a rare, life-threatening syndrome characterized by occlusion of microvessels in the subcutaneous adipose tissue and dermis, in addition to other tissues, resulting in extremely painful ischemic lesions. However, its pathophysiology is still poorly understood. In any case, the analysis of risk factors associated with it allows us to identify possible pathophysiological mechanisms. […] Calciphylaxis is associated with the use of vitamin K inhibitors. This inhibition prevents the activation of matrix Gla protein (MGP), an extracellular matrix protein synthesized in the endothelial, vascular smooth muscle and in the chondrocytes. It is a potent inhibitor of calcification. Other conditions associated with vitamin K deficiency are also risk factors for the onset of calciphylaxis, such as liver disease, gastric bypass, and obesity.

• #7 Calciphylaxis

  https://mobile.fpnotebook.com/Renal/Derm/Clcphylxs.htm

  Intra-arteriole thrombus may be present. […] Dermal and epidermal ulcerations and necrosis. […] […] […] Calciphylaxis is often a clinical diagnosis that does not require skin biopsy. […] Under-recognized condition outside of Hemodialysis centers (missed diagnosis is not uncommon). […] Very high morbidity and mortality. […] […] […] Skin ischemia may progress to skin necrosis. […] One year survival is 50%. […] Mortality typically due to Sepsis.

• #8 Calciphylaxis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/calciphylaxis?lang=us

  Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare condition that manifests as subcutaneous vascular calcification and cutaneous necrosis (small blood vessels of the fat tissue and the skin). Some authors describe it as a syndrome of vascular calcification, thrombosis and skin necrosis. […] The exact pathogenesis of calciphylaxis is unclear. Medial calcification and intimal fibrosis of the cutaneous arterioles combined with thrombotic occlusion leading to ischemic skin necrosis is seen in calciphylaxis. […] More than 50 percent of patients die (most commonly from sepsis) within one year of being diagnosed.

• #9 Calciphylaxis Diagnosis and Management in Primary Care

  https://www.clinicaladvisor.com/features/calciphylaxis-diagnosis-treatment-primary-care/

  Calciphylaxis should be considered in patients presenting with painful nonhealing skin ulcers who have chronic kidney disease, obesity, have been on hemodialysis, and/or are of White race and female sex. Skin changes associated with calciphylaxis have sudden onset and progress rapidly to open ulcerations and then necrosis. A thorough physical examination of the patient, preferably with the torso and lower extremities exposed, can assist the clinician in visualizing the patients lesions and assessing skin integrity. A painful necrotic ulcer covered with black eschar that started as painful ecchymoses is usually the presenting symptom; patients often mistake initial reddening for a bruise and delay seeking medical care. The presence of malodorous, dusky, and/or necrotic lesions should increase suspicion of calciphylaxis. Clinical findings include purple or red net-like areas of ecchymoses that are painful or nonhealing ulcers that are painful. Imaging studies such as radiograph and bone scan may support the diagnosis by allowing for determination of areas of microcalcifications in calciphylaxis. A positive bone scan has a high sensitivity rate of 97% in cases of calciphylaxis. Laboratory tests such as renal function tests, serum calcium, alkaline phosphatase, phosphorous, and vitamin D can evaluate potential risk factors. The gold standard for diagnosing calciphylaxis is skin biopsy. Clinicians with adequate training should perform the skin biopsy to prevent further trauma to skin that is already fragile and difficult to heal. Clinicians must weigh the benefits and risks of biopsy; patients with ESRD and classic presentation of calciphylaxis do not require biopsy. The double-punch technique, where a punch biopsy is followed by a second punch inserted through the center of the first punch, can offer an improved yield on the composition of the skin and underlying structures. Once diagnosis of calciphylaxis is made, referral to a dermatologist is helpful for management of the damaged skin.

• #10 Calciphylaxis: Approach to Diagnosis and Management – Making the Rounds

  https://internalmedicineiowa.org/2020/01/13/calciphylaxis-approach-to-diagnosis-and-management/

  Calciphylaxis is a rare disorder of poor prognosis that can lead to intense, painful lesions involving the skin and subcutaneous tissue. […] The diagnosis of calciphylaxis is complicated by the absence of a gold standard marker of disease such as a clear histopathological finding. […] Late diagnosis and advanced lesions can significantly shorten life expectancy. […] Future research is required to establish clear causal pathways and improve on the treatment options currently available to patients.

• #11 SciELO Brazil – Diagnosis and treatment of calciphylaxis in patients with chronic kidney disease Diagnosis and treatment of calciphylaxis in patients with chronic kidney disease

  https://www.scielo.br/j/jbn/a/RVkm7hnwxgvvh8NhdWK9CYy/

  1. The diagnosis of calciphylaxis is clinical. Calciphylaxis should be suspected in CKD patients presenting with nodular, purpuric/erythematous lesions or painful subcutaneous plaques, livedo reticularis, non-healing ulcers and/or skin necrosis, especially on the thighs and other areas of increased adiposity (Evidence). […] The diagnosis of calciphylaxis is clinical. Patients with dialysis or non-dialysis CKD presenting with complaints of severe pain (usually stabbing), skin lesions and subcutaneous induration on palpation have calciphylaxis, until proven otherwise. If subjected to skin biopsy, the main histological findings are: small vessels calcification, intimal hyperplasia and thrombosis of microvessels in adipose, subcutaneous tissue and dermis. Calcified lesions are composed of calcium and phosphorus. Inflammatory infiltrate is often observed. Arterial calcification, associated with the destruction of the endothelium and thrombosis leads to clinical manifestations of calciphylaxis. Specific stains to highlight the presence of calcium in the tissue, as the Von Kossa stain, are important for complementing the diagnosis (Evidence).

• #12 Calciphylaxis and its diagnosis: A review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6820424/

  Calciphylaxis also known as Calcific uremic arteriolopathy (CUA), is a rare fatal complication usually associated with end-stage renal disease (ESRD). […] Skin biopsy and radiographic features are helpful in the diagnosis of calciphylaxis, but negative results do not necessarily exclude the diagnosis. This article highlights steps undertaking in the diagnosis of calciphylaxis. […] Intense pain associated with palpation of firm calcified subcutaneous tissue and cutaneous lesions is suggestive of calciphylaxis in dialysis patients and in patients with other risk factors for the disorder. […] Various steps that are undertaken for accurate diagnosis of the disease are as follows. […] The clinical examination of a patient with calciphylaxis involves two important goals: to evaluate the presence of any etiological factor and to rule out any potential disorders that may mimic the physical examination findings.

• #13 Calciphylaxis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK519020/

  Calciphylaxis typically presents with extremely painful ischemic cutaneous lesions or painful subcutaneous nodules without skin changes, although at times, pain may precede the development of the lesions. […] Diagnosing calciphylaxis requires a high index of suspicion. A definitive diagnosis is made after a skin biopsy of one of the lesions. The histologic evaluation shows medial calcification of dermal arterioles or small arteries and may show fibrointimal hyperplasia, microthrombi, and vascular narrowing or occlusion, often with evidence of necrosis. […] The need for biopsy in the diagnosis of calciphylaxis is debatable. While a biopsy is often performed to differentiate early stages of calciphylaxis from other skin lesions, the biopsy itself carries some risk, particularly for patients with only subcutaneous nodules.

• #14 Calciphylaxis and its diagnosis: A review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6820424/

  Calciphylaxis also known as Calcific uremic arteriolopathy (CUA), is a rare fatal complication usually associated with end-stage renal disease (ESRD). […] Skin biopsy and radiographic features are helpful in the diagnosis of calciphylaxis, but negative results do not necessarily exclude the diagnosis. This article highlights steps undertaking in the diagnosis of calciphylaxis. […] Intense pain associated with palpation of firm calcified subcutaneous tissue and cutaneous lesions is suggestive of calciphylaxis in dialysis patients and in patients with other risk factors for the disorder. […] Various steps that are undertaken for accurate diagnosis of the disease are as follows. […] The clinical examination of a patient with calciphylaxis involves two important goals: to evaluate the presence of any etiological factor and to rule out any potential disorders that may mimic the physical examination findings.

• #15 Calciphylaxis Diagnosis and Management in Primary Care

  https://www.renalandurologynews.com/features/calciphylaxis-diagnosis-treatment-primary-care-2/

  Calciphylaxis should be considered in patients presenting with painful nonhealing skin ulcers who have chronic kidney disease, obesity, have been on hemodialysis, and/or are of White race and female sex. Skin changes associated with calciphylaxis have sudden onset and progress rapidly to open ulcerations and then necrosis. A thorough physical examination of the patient, preferably with the torso and lower extremities exposed, can assist the clinician in visualizing the patients lesions and assessing skin integrity. A painful necrotic ulcer covered with black eschar that started as painful ecchymoses is usually the presenting symptom; patients often mistake initial reddening for a bruise and delay seeking medical care. The presence of malodorous, dusky, and/or necrotic lesions should increase suspicion of calciphylaxis. Clinical findings include purple or red net-like areas of ecchymoses that are painful or nonhealing ulcers that are painful. Imaging studies such as radiograph and bone scan may support the diagnosis by allowing for determination of areas of microcalcifications in calciphylaxis. A positive bone scan has a high sensitivity rate of 97% in cases of calciphylaxis. The gold standard for diagnosing calciphylaxis is skin biopsy. Clinicians with adequate training should perform the skin biopsy to prevent further trauma to skin that is already fragile and difficult to heal. Clinicians must weigh the benefits and risks of biopsy; patients with ESRD and classic presentation of calciphylaxis do not require biopsy. The double-punch technique, where a punch biopsy is followed by a second punch inserted through the center of the first punch, can offer an improved yield on the composition of the skin and underlying structures. Once diagnosis of calciphylaxis is made, referral to a dermatologist is helpful for management of the damaged skin.

• #16 Calciphylaxis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK519020/

  Calciphylaxis typically presents with extremely painful ischemic cutaneous lesions or painful subcutaneous nodules without skin changes, although at times, pain may precede the development of the lesions. […] Diagnosing calciphylaxis requires a high index of suspicion. A definitive diagnosis is made after a skin biopsy of one of the lesions. The histologic evaluation shows medial calcification of dermal arterioles or small arteries and may show fibrointimal hyperplasia, microthrombi, and vascular narrowing or occlusion, often with evidence of necrosis. […] The need for biopsy in the diagnosis of calciphylaxis is debatable. While a biopsy is often performed to differentiate early stages of calciphylaxis from other skin lesions, the biopsy itself carries some risk, particularly for patients with only subcutaneous nodules.

• #17 Calciphylaxis Diagnosis and Management in Primary Care

  https://www.clinicaladvisor.com/features/calciphylaxis-diagnosis-treatment-primary-care/

  Calciphylaxis should be considered in patients presenting with painful nonhealing skin ulcers who have chronic kidney disease, obesity, have been on hemodialysis, and/or are of White race and female sex. Skin changes associated with calciphylaxis have sudden onset and progress rapidly to open ulcerations and then necrosis. A thorough physical examination of the patient, preferably with the torso and lower extremities exposed, can assist the clinician in visualizing the patients lesions and assessing skin integrity. A painful necrotic ulcer covered with black eschar that started as painful ecchymoses is usually the presenting symptom; patients often mistake initial reddening for a bruise and delay seeking medical care. The presence of malodorous, dusky, and/or necrotic lesions should increase suspicion of calciphylaxis. Clinical findings include purple or red net-like areas of ecchymoses that are painful or nonhealing ulcers that are painful. Imaging studies such as radiograph and bone scan may support the diagnosis by allowing for determination of areas of microcalcifications in calciphylaxis. A positive bone scan has a high sensitivity rate of 97% in cases of calciphylaxis. Laboratory tests such as renal function tests, serum calcium, alkaline phosphatase, phosphorous, and vitamin D can evaluate potential risk factors. The gold standard for diagnosing calciphylaxis is skin biopsy. Clinicians with adequate training should perform the skin biopsy to prevent further trauma to skin that is already fragile and difficult to heal. Clinicians must weigh the benefits and risks of biopsy; patients with ESRD and classic presentation of calciphylaxis do not require biopsy. The double-punch technique, where a punch biopsy is followed by a second punch inserted through the center of the first punch, can offer an improved yield on the composition of the skin and underlying structures. Once diagnosis of calciphylaxis is made, referral to a dermatologist is helpful for management of the damaged skin.

• #18 Calciphylaxis Diagnosis and Management in Primary Care

  https://www.clinicaladvisor.com/features/calciphylaxis-diagnosis-treatment-primary-care/

  Calciphylaxis should be considered in patients presenting with painful nonhealing skin ulcers who have chronic kidney disease, obesity, have been on hemodialysis, and/or are of White race and female sex. Skin changes associated with calciphylaxis have sudden onset and progress rapidly to open ulcerations and then necrosis. A thorough physical examination of the patient, preferably with the torso and lower extremities exposed, can assist the clinician in visualizing the patients lesions and assessing skin integrity. A painful necrotic ulcer covered with black eschar that started as painful ecchymoses is usually the presenting symptom; patients often mistake initial reddening for a bruise and delay seeking medical care. The presence of malodorous, dusky, and/or necrotic lesions should increase suspicion of calciphylaxis. Clinical findings include purple or red net-like areas of ecchymoses that are painful or nonhealing ulcers that are painful. Imaging studies such as radiograph and bone scan may support the diagnosis by allowing for determination of areas of microcalcifications in calciphylaxis. A positive bone scan has a high sensitivity rate of 97% in cases of calciphylaxis. Laboratory tests such as renal function tests, serum calcium, alkaline phosphatase, phosphorous, and vitamin D can evaluate potential risk factors. The gold standard for diagnosing calciphylaxis is skin biopsy. Clinicians with adequate training should perform the skin biopsy to prevent further trauma to skin that is already fragile and difficult to heal. Clinicians must weigh the benefits and risks of biopsy; patients with ESRD and classic presentation of calciphylaxis do not require biopsy. The double-punch technique, where a punch biopsy is followed by a second punch inserted through the center of the first punch, can offer an improved yield on the composition of the skin and underlying structures. Once diagnosis of calciphylaxis is made, referral to a dermatologist is helpful for management of the damaged skin.

• #19 What Is Your Diagnosis? Calciphylaxis | MDedge

  https://community.the-hospitalist.org/content/what-your-diagnosis-calciphylaxis

  Calciphylaxis is a rare disorder that most commonly affects patients with impaired renal failure. Calciphylaxis, known as calcific uremic arteriolopathy, involves calcification of small- and medium-sized blood vessels, which may lead to necrosis of the dermis, subcutaneous tissue, muscles, fascia, and internal organs. Calciphylaxis usually occurs in fatty areas such as the calves, thighs, abdomen, and buttocks. Although calciphylaxis most commonly is associated with severe renal disease, pharmacologic agents such as glucocorticoids, high-dose vitamin D, warfarin, iron salts, insulin injections, calcium-based phosphate binders, and chemotherapeutic agents also may precipitate this condition. Diagnostic biopsies typically reveal fat necrosis, inflammation, and associated small vessel calcification.

• #20 Calciphylaxis Diagnosis and Management in Primary Care

  https://www.clinicaladvisor.com/features/calciphylaxis-diagnosis-treatment-primary-care/

  Calciphylaxis should be considered in patients presenting with painful nonhealing skin ulcers who have chronic kidney disease, obesity, have been on hemodialysis, and/or are of White race and female sex. Skin changes associated with calciphylaxis have sudden onset and progress rapidly to open ulcerations and then necrosis. A thorough physical examination of the patient, preferably with the torso and lower extremities exposed, can assist the clinician in visualizing the patients lesions and assessing skin integrity. A painful necrotic ulcer covered with black eschar that started as painful ecchymoses is usually the presenting symptom; patients often mistake initial reddening for a bruise and delay seeking medical care. The presence of malodorous, dusky, and/or necrotic lesions should increase suspicion of calciphylaxis. Clinical findings include purple or red net-like areas of ecchymoses that are painful or nonhealing ulcers that are painful. Imaging studies such as radiograph and bone scan may support the diagnosis by allowing for determination of areas of microcalcifications in calciphylaxis. A positive bone scan has a high sensitivity rate of 97% in cases of calciphylaxis. Laboratory tests such as renal function tests, serum calcium, alkaline phosphatase, phosphorous, and vitamin D can evaluate potential risk factors. The gold standard for diagnosing calciphylaxis is skin biopsy. Clinicians with adequate training should perform the skin biopsy to prevent further trauma to skin that is already fragile and difficult to heal. Clinicians must weigh the benefits and risks of biopsy; patients with ESRD and classic presentation of calciphylaxis do not require biopsy. The double-punch technique, where a punch biopsy is followed by a second punch inserted through the center of the first punch, can offer an improved yield on the composition of the skin and underlying structures. Once diagnosis of calciphylaxis is made, referral to a dermatologist is helpful for management of the damaged skin.

• #21 Calciphylaxis in patients with chronic kidney disease: A disease which is still bewildering and potentially fatal | Nefrología

  https://www.revistanefrologia.com/en-calciphylaxis-in-patients-with-chronic-articulo-resumen-S2013251418301068

  Recently, there have been proposed diagnostic criteria, that have some limitations but these are the only one available. According to these criteria, the diagnosis of calciphylaxis can be made if the three following clinical features are present: (1) patient on hemodialysis or with glomerular filtration […] (2) with two painful and non-curable ulcers with associated painful purpura (3) present in the trunk, extremities or penis. […] The most reliable diagnostic technique is skin biopsy, although often the clinical presentation may be sufficient to make the diagnosis.

• #22 Calciphylaxis Diagnosis and Management in Primary Care

  https://www.clinicaladvisor.com/features/calciphylaxis-diagnosis-treatment-primary-care/

  Calciphylaxis should be considered in patients presenting with painful nonhealing skin ulcers who have chronic kidney disease, obesity, have been on hemodialysis, and/or are of White race and female sex. Skin changes associated with calciphylaxis have sudden onset and progress rapidly to open ulcerations and then necrosis. A thorough physical examination of the patient, preferably with the torso and lower extremities exposed, can assist the clinician in visualizing the patients lesions and assessing skin integrity. A painful necrotic ulcer covered with black eschar that started as painful ecchymoses is usually the presenting symptom; patients often mistake initial reddening for a bruise and delay seeking medical care. The presence of malodorous, dusky, and/or necrotic lesions should increase suspicion of calciphylaxis. Clinical findings include purple or red net-like areas of ecchymoses that are painful or nonhealing ulcers that are painful. Imaging studies such as radiograph and bone scan may support the diagnosis by allowing for determination of areas of microcalcifications in calciphylaxis. A positive bone scan has a high sensitivity rate of 97% in cases of calciphylaxis. Laboratory tests such as renal function tests, serum calcium, alkaline phosphatase, phosphorous, and vitamin D can evaluate potential risk factors. The gold standard for diagnosing calciphylaxis is skin biopsy. Clinicians with adequate training should perform the skin biopsy to prevent further trauma to skin that is already fragile and difficult to heal. Clinicians must weigh the benefits and risks of biopsy; patients with ESRD and classic presentation of calciphylaxis do not require biopsy. The double-punch technique, where a punch biopsy is followed by a second punch inserted through the center of the first punch, can offer an improved yield on the composition of the skin and underlying structures. Once diagnosis of calciphylaxis is made, referral to a dermatologist is helpful for management of the damaged skin.

• #23 Calciphylaxis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK519020/

  Calciphylaxis typically presents with extremely painful ischemic cutaneous lesions or painful subcutaneous nodules without skin changes, although at times, pain may precede the development of the lesions. […] Diagnosing calciphylaxis requires a high index of suspicion. A definitive diagnosis is made after a skin biopsy of one of the lesions. The histologic evaluation shows medial calcification of dermal arterioles or small arteries and may show fibrointimal hyperplasia, microthrombi, and vascular narrowing or occlusion, often with evidence of necrosis. […] The need for biopsy in the diagnosis of calciphylaxis is debatable. While a biopsy is often performed to differentiate early stages of calciphylaxis from other skin lesions, the biopsy itself carries some risk, particularly for patients with only subcutaneous nodules.

• #24 Advanced-stage calciphylaxis: Think before you punch | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/83/8/562

  In the advanced stages, the diagnosis of calciphylaxis is clinically more evident, and the differential diagnosis usually narrows. […] Well-demarcated, necrotic, indurated lesions that are bilateral in a patient with end-stage renal disease without shock makes the diagnosis very likely. […] Biopsy for histologic confirmation of calciphylaxis can increase the risk of infection and sepsis. […] Since extensive necrosis predisposes to a negative biopsy, a high clinical suspicion should drive early treatment of calciphylaxis. […] Noninvasive imaging studies such as plain radiography and bone scintigraphy can aid the diagnosis by detecting moderate to severe soft-tissue vascular calcification in these areas.

• #25

  https://journals.lww.com/jfmpc/fulltext/2019/08090/calciphylaxis_and_its_diagnosis__a_review.4.aspx

  Even plain X-rays, nuclear bone scans and circulating fetuin A levels have been reported to aid in the diagnosis, none of these tools have been recommended for clinical use. […] The main requirement in this diagnostic procedure is of a fiber-optic handheld probe that detects the carbonated apatite in calciphylaxis at various sites and depths. […] Best way to confirm calciphylaxis requires biopsy of involved area of skin and the test should be performed whenever the diagnosis is considered. […] Lesional biopsy can demonstrate medial calcification and intimal proliferation of small vessels. Other diagnostic histopathological features are extravascular soft tissue calcification, septal and lobular panniculitis, dermal-epidermal split, and epidermal necrosis. […] The diagnostic criteria based on biopsy sometimes indicate low sensitivity in patients where biopsy specimen lacks calcifications.

• #26 Advanced-stage calciphylaxis: Think before you punch | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/83/8/562

  A 53-year-old woman presented with extensive, nonulcerated, painful plaques on both calves. […] Histologic study of a full-thickness skin biopsy specimen showed tissue necrosis, ulceration, and concentric calcification of small and medium-sized blood vessels, many with luminal thrombi, all of which together were diagnostic for calciphylaxis. […] There are no strict guidelines for the diagnosis of calciphylaxis, and the exact pathophysiology of calciphylaxis is not understood. […] Ulceration is considered the clinical hallmark, but there are increasing reports of patients presenting with nonulcerated plaques, as in our patient. […] Histologic features identified on full-thickness biopsy specimens are intravascular deposition of calcium in the media of the blood vessels, as well as fibrin thrombi formation, intimal proliferation, tissue necrosis, and resultant ischemia.

• #27 Calciphylaxis Workup: Laboratory Studies, Imaging Studies, Procedures

  https://emedicine.medscape.com/article/1095481-workup

  In the workup for calciphylaxis (also referred to as calcific uremic arteriolopathy), the following laboratory measurements may be considered: […] Plain radiography uniformly demonstrates an arborization of vascular calcification within the dermis and the subcutaneous tissue. […] A study by Shmidt et al showed that patients with calciphylaxis had more vascular calcifications, more small-vessel calcifications, and a netlike pattern of calcifications. This netlike pattern, when present, was strongly associated with the presence of calciphylaxis. […] Bone scintigraphy may be used as a noninvasive diagnostic tool because the bone matrix protein osteopontin has been demonstrated in calciphylaxis lesions. […] Biopsy specimens typically demonstrate calcification within the media and intima of small and medium-sized arterioles with extensive intimal hyperplasia and fibrosis, but this also may be seen in uninvolved skin in patients with chronic kidney disease (CKD; or chronic renal failure) or atherosclerosis. […] The decision to perform a biopsy on a nonulcerated lesion should not be made lightly, because it could result in a nonhealing wound. […] Although calciphylaxis lesions have a clinical appearance suggestive of avascular necrosis (AVN), the tissue often bleeds freely during surgery.

• #28 Calciphylaxis and its diagnosis: A review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6820424/

  Even plain X-rays, nuclear bone scans and circulating fetuin A levels have been reported to aid in the diagnosis, none of these tools have been recommended for clinical use. […] The best way to confirm calciphylaxis requires biopsy of involved area of skin and the test should be performed whenever the diagnosis is considered. […] Lesional biopsy can demonstrate medial calcification and intimal proliferation of small vessels. Other diagnostic histopathological features are extravascular soft tissue calcification, septal and lobular panniculitis, dermal-epidermal split, and epidermal necrosis. […] The diagnostic criteria based on biopsy sometimes indicate low sensitivity in patients where biopsy specimen lacks calcifications. […] Further for obtaining sufficient biopsy sample an excisional biopsy or punch biopsy with a depth of 6-8 mm can be done. If necessary, at the base of large punch biopsy a telescoping 4 mm punch biopsy is done for obtaining sufficient tissue.

• #29 Role of imaging in calciphylaxis diagnosis | Eurorad

  https://www.eurorad.org/case/16949

  0 Role of imaging in calciphylaxis diagnosis […] The role of radiology in the diagnosis is not well established. Different imaging modalities can detect extraosseous calcium deposition. […] Mammography can detect calcification in arteries as small as 0,13mm in calibre and provides a better resolution than conventional X-Ray or CT scan. […] Calcification of small arterioles in a netlike pattern have been described as the key imaging feature and it is highly suggestive of the diagnosis in symptomatic patients […] Nuclear medicine techniques such as bone scintigraphy using Tc99 have a high sensitivity in detection of extraosseous calcifications and can assess both extent as well as presence of active disease. […] Final diagnosis requires a skin biopsy showing characteristic histopathological features including medial calcification, intimal proliferation and microthrombi in small arteries. Extravascular interstitial calcium deposition, especially in perieccrine location, as well as resultant ischemic necrosis and inflammatory changes also help establish the diagnosis.

• #30

  https://journals.lww.com/jfmpc/fulltext/2019/08090/calciphylaxis_and_its_diagnosis__a_review.4.aspx

  Even plain X-rays, nuclear bone scans and circulating fetuin A levels have been reported to aid in the diagnosis, none of these tools have been recommended for clinical use. […] The main requirement in this diagnostic procedure is of a fiber-optic handheld probe that detects the carbonated apatite in calciphylaxis at various sites and depths. […] Best way to confirm calciphylaxis requires biopsy of involved area of skin and the test should be performed whenever the diagnosis is considered. […] Lesional biopsy can demonstrate medial calcification and intimal proliferation of small vessels. Other diagnostic histopathological features are extravascular soft tissue calcification, septal and lobular panniculitis, dermal-epidermal split, and epidermal necrosis. […] The diagnostic criteria based on biopsy sometimes indicate low sensitivity in patients where biopsy specimen lacks calcifications.

• #31 Calciphylaxis and its diagnosis: A review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6820424/

  Even plain X-rays, nuclear bone scans and circulating fetuin A levels have been reported to aid in the diagnosis, none of these tools have been recommended for clinical use. […] The best way to confirm calciphylaxis requires biopsy of involved area of skin and the test should be performed whenever the diagnosis is considered. […] Lesional biopsy can demonstrate medial calcification and intimal proliferation of small vessels. Other diagnostic histopathological features are extravascular soft tissue calcification, septal and lobular panniculitis, dermal-epidermal split, and epidermal necrosis. […] The diagnostic criteria based on biopsy sometimes indicate low sensitivity in patients where biopsy specimen lacks calcifications. […] Further for obtaining sufficient biopsy sample an excisional biopsy or punch biopsy with a depth of 6-8 mm can be done. If necessary, at the base of large punch biopsy a telescoping 4 mm punch biopsy is done for obtaining sufficient tissue.

• #32

  https://journals.lww.com/jfmpc/fulltext/2019/08090/calciphylaxis_and_its_diagnosis__a_review.4.aspx

  Even plain X-rays, nuclear bone scans and circulating fetuin A levels have been reported to aid in the diagnosis, none of these tools have been recommended for clinical use. […] The main requirement in this diagnostic procedure is of a fiber-optic handheld probe that detects the carbonated apatite in calciphylaxis at various sites and depths. […] Best way to confirm calciphylaxis requires biopsy of involved area of skin and the test should be performed whenever the diagnosis is considered. […] Lesional biopsy can demonstrate medial calcification and intimal proliferation of small vessels. Other diagnostic histopathological features are extravascular soft tissue calcification, septal and lobular panniculitis, dermal-epidermal split, and epidermal necrosis. […] The diagnostic criteria based on biopsy sometimes indicate low sensitivity in patients where biopsy specimen lacks calcifications.

• #33 Calciphylaxis and its diagnosis: A review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6820424/

  Even plain X-rays, nuclear bone scans and circulating fetuin A levels have been reported to aid in the diagnosis, none of these tools have been recommended for clinical use. […] The best way to confirm calciphylaxis requires biopsy of involved area of skin and the test should be performed whenever the diagnosis is considered. […] Lesional biopsy can demonstrate medial calcification and intimal proliferation of small vessels. Other diagnostic histopathological features are extravascular soft tissue calcification, septal and lobular panniculitis, dermal-epidermal split, and epidermal necrosis. […] The diagnostic criteria based on biopsy sometimes indicate low sensitivity in patients where biopsy specimen lacks calcifications. […] Further for obtaining sufficient biopsy sample an excisional biopsy or punch biopsy with a depth of 6-8 mm can be done. If necessary, at the base of large punch biopsy a telescoping 4 mm punch biopsy is done for obtaining sufficient tissue.

• #34

  https://journals.lww.com/aswcjournal/fulltext/2019/05000/calciphylaxis__diagnosis,_pathogenesis,_and.3.aspx

  A punch biopsy with a double trephine technique is the preferred biopsy method. […] Bone scan may prove to be a reliable, noninvasive diagnostic test for calciphylaxis, especially when biopsy findings are nondiagnostic or when a biopsy cannot be performed. […] Laboratory evaluation is diagnostically unhelpful.

• #35

  https://journals.lww.com/jfmpc/fulltext/2019/08090/calciphylaxis_and_its_diagnosis__a_review.4.aspx

  Even plain X-rays, nuclear bone scans and circulating fetuin A levels have been reported to aid in the diagnosis, none of these tools have been recommended for clinical use. […] The main requirement in this diagnostic procedure is of a fiber-optic handheld probe that detects the carbonated apatite in calciphylaxis at various sites and depths. […] Best way to confirm calciphylaxis requires biopsy of involved area of skin and the test should be performed whenever the diagnosis is considered. […] Lesional biopsy can demonstrate medial calcification and intimal proliferation of small vessels. Other diagnostic histopathological features are extravascular soft tissue calcification, septal and lobular panniculitis, dermal-epidermal split, and epidermal necrosis. […] The diagnostic criteria based on biopsy sometimes indicate low sensitivity in patients where biopsy specimen lacks calcifications.

• #36 Calciphylaxis Diagnosis and Management in Primary Care

  https://www.clinicaladvisor.com/features/calciphylaxis-diagnosis-treatment-primary-care/

  Calciphylaxis should be considered in patients presenting with painful nonhealing skin ulcers who have chronic kidney disease, obesity, have been on hemodialysis, and/or are of White race and female sex. Skin changes associated with calciphylaxis have sudden onset and progress rapidly to open ulcerations and then necrosis. A thorough physical examination of the patient, preferably with the torso and lower extremities exposed, can assist the clinician in visualizing the patients lesions and assessing skin integrity. A painful necrotic ulcer covered with black eschar that started as painful ecchymoses is usually the presenting symptom; patients often mistake initial reddening for a bruise and delay seeking medical care. The presence of malodorous, dusky, and/or necrotic lesions should increase suspicion of calciphylaxis. Clinical findings include purple or red net-like areas of ecchymoses that are painful or nonhealing ulcers that are painful. Imaging studies such as radiograph and bone scan may support the diagnosis by allowing for determination of areas of microcalcifications in calciphylaxis. A positive bone scan has a high sensitivity rate of 97% in cases of calciphylaxis. Laboratory tests such as renal function tests, serum calcium, alkaline phosphatase, phosphorous, and vitamin D can evaluate potential risk factors. The gold standard for diagnosing calciphylaxis is skin biopsy. Clinicians with adequate training should perform the skin biopsy to prevent further trauma to skin that is already fragile and difficult to heal. Clinicians must weigh the benefits and risks of biopsy; patients with ESRD and classic presentation of calciphylaxis do not require biopsy. The double-punch technique, where a punch biopsy is followed by a second punch inserted through the center of the first punch, can offer an improved yield on the composition of the skin and underlying structures. Once diagnosis of calciphylaxis is made, referral to a dermatologist is helpful for management of the damaged skin.

• #37 Calciphylaxis: Symptoms & Causes

  https://my.clevelandclinic.org/health/diseases/22359-calciphylaxis

  A healthcare provider may suspect calciphylaxis based on your condition, symptoms and a physical exam of your body. This exam includes looking and feeling for any changes to your skin or the area just underneath it. Theyll also ask questions about your medical history as they try to make a calciphylaxis diagnosis. Once a provider suspects calciphylaxis, theyll order medical tests to learn more. […] A healthcare provider may order the following tests: […] Taking a sample of skin and tissue just underneath (usually about 4 or 5 millimeters deep) is the most reliable way to identify calciphylaxis. A provider usually takes a sample at the edge of a lesion or wound. Analyzing the sample involves looking at it under a microscope and using certain substances to cause color or chemical changes that can help confirm the diagnosis.

• #38 Calciphylaxis Diagnosis and Management in Primary Care

  https://www.clinicaladvisor.com/features/calciphylaxis-diagnosis-treatment-primary-care/

  Calciphylaxis should be considered in patients presenting with painful nonhealing skin ulcers who have chronic kidney disease, obesity, have been on hemodialysis, and/or are of White race and female sex. Skin changes associated with calciphylaxis have sudden onset and progress rapidly to open ulcerations and then necrosis. A thorough physical examination of the patient, preferably with the torso and lower extremities exposed, can assist the clinician in visualizing the patients lesions and assessing skin integrity. A painful necrotic ulcer covered with black eschar that started as painful ecchymoses is usually the presenting symptom; patients often mistake initial reddening for a bruise and delay seeking medical care. The presence of malodorous, dusky, and/or necrotic lesions should increase suspicion of calciphylaxis. Clinical findings include purple or red net-like areas of ecchymoses that are painful or nonhealing ulcers that are painful. Imaging studies such as radiograph and bone scan may support the diagnosis by allowing for determination of areas of microcalcifications in calciphylaxis. A positive bone scan has a high sensitivity rate of 97% in cases of calciphylaxis. Laboratory tests such as renal function tests, serum calcium, alkaline phosphatase, phosphorous, and vitamin D can evaluate potential risk factors. The gold standard for diagnosing calciphylaxis is skin biopsy. Clinicians with adequate training should perform the skin biopsy to prevent further trauma to skin that is already fragile and difficult to heal. Clinicians must weigh the benefits and risks of biopsy; patients with ESRD and classic presentation of calciphylaxis do not require biopsy. The double-punch technique, where a punch biopsy is followed by a second punch inserted through the center of the first punch, can offer an improved yield on the composition of the skin and underlying structures. Once diagnosis of calciphylaxis is made, referral to a dermatologist is helpful for management of the damaged skin.

• #39

  https://journals.lww.com/aswcjournal/fulltext/2019/05000/calciphylaxis__diagnosis,_pathogenesis,_and.3.aspx

  To provide information on the pathogenesis, clinical features, diagnosis, and treatment of calciphylaxis. […] Explain the diagnosis and management of a patient with calciphylaxis. […] Diagnosis can be made on clinical grounds alone when a patient with ESRD presents with indurated tender plaques or ulcers on the abdomen and/or legs. […] Skin biopsy may be necessary to differentiate calciphylaxis from its mimics (eg, pyoderma gangrenosum, Martorell hypertensive ischemic leg ulcer, cholesterol emboli, etc; Table 2), especially when calciphylaxis presents in early stages with clinically nondescript lesions such as a small papule and erosion. […] In contrast, skin biopsy is not always indicated in uremic calciphylaxis because the likelihood of calciphylaxis is much higher when an ESRD patient presents with characteristic skin lesions, as compared with patients with preserved renal function.

• #40

  https://link.springer.com/article/10.1007/s12325-020-01504-w

  The characteristic feature of calciphylaxis is the diffuse calcification of small capillaries in adipose tissue. […] In certain cases, medical imaging can be helpful in making a diagnosis. […] The current literature shows that serum laboratory investigations yield little in the way of definitive proof in calciphylaxis cases. […] Cutaneous calciphylaxis can mimic many other skin conditions and diagnosis can be difficult to make, especially in non-uremic patients. Currently, skin biopsy with histological examination is the best method of diagnosing the disease, with medical imaging being helpful in select cases.

• #41 Calciphylaxis – Wikipedia

  https://en.wikipedia.org/wiki/Calciphylaxis

  Calciphylaxis is characterized by the following histologic findings: […] The diagnosis is a clinical one. The characteristic lesions are the ischemic skin lesions (usually with areas of skin necrosis). The necrotic skin lesions (i.e. the dying or already dead skin areas) typically appear as violaceous (dark bluish purple) lesions and/or completely black leathery lesions. […] The suspected diagnosis can be supported by a skin biopsy, usually a punch biopsy, which shows arterial calcification and occlusion in the absence of vasculitis. […] Laboratory studies, such as phosphate levels, calcium levels, and parathyroid levels, are nonspecific and unhelpful for diagnosis of calciphylaxis.

• #42 Calciphylaxis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/calciphylaxis/diagnosis-treatment/drc-20370562

  Diagnosis involves finding out if calciphylaxis is the cause of your symptoms. Your healthcare professional reviews your health history, asks about your symptoms and gives you a physical exam. […] You also may need tests such as: […] Skin biopsy. During this procedure, your healthcare professional removes a small tissue sample from an area of affected skin. Then, a lab checks the sample. […] Blood tests. A lab can measure various substances in your blood. These include creatinine, calcium, phosphorus, parathyroid hormone and vitamin D. The results help your healthcare team check how well your kidneys are working. […] Imaging tests. These can be useful if biopsy results aren’t clear or if a biopsy can’t be done. X-rays may show calcium buildups in the blood vessels. These buildups are common in calciphylaxis and in other advanced kidney diseases.

• #43 Calciphylaxis: Pictures, Definition, Symptoms, Treatment, and Outlook

  https://www.healthline.com/health/calciphylaxis

  Calciphylaxis is a kidney complication causing calcium buildup inside fat and skin blood vessels. […] Although a rare condition, if you suspect you have calciphylaxis, do not hesitate to contact your doctor. Early treatment may improve your outlook. […] A doctor might suspect calciphylaxis based on the presence of painful skin lesions. If such physical symptoms are present, they’ll perform a physical examination or skin biopsy both important tools to help confirm calciphylaxis. […] Your doctor might also run several tests to rule out other complications of chronic kidney disease. Some diagnostic tests may include: skin biopsy, blood tests for levels of calcium, phosphorous, alkaline phosphatase, PTH, 25-hydroxyvitamin D, liver function blood tests, kidney function tests, tests to evaluate infections, such as a complete blood count and blood culture tests.

• #44 SSA – POMS: DI 23022.128 – Calciphylaxis – 08/09/2023

  https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022128

  Calciphylaxis is a rare metabolic disease in which calcium builds up in the walls of veins and arteries, blocking blood flow and resulting in damage to multiple organ systems. […] A diagnosis of calciphylaxis can be confirmed through skin biopsy, function tests of the kidneys and liver, and tests for concentration of calcium and other minerals in the blood. […] In some advanced cases involving ESRD, diagnosis is possible through visual inspection of wounds. […] Suggested MER for Evaluation: Clinical history and examination that describes the diagnostic features of the impairment; and Laboratory tests confirming impaired liver and kidney function, and threshold levels of calcium on fat and blood.

• #45 Calciphylaxis Prevention and Treatment Strategies- Clinical Advisor

  https://www.clinicaladvisor.com/features/calciphylaxis-prevention-treatment-strategies/

  Diagnosis can be made clinically, although skin biopsy and histology may help in the diagnosis. Histologic evidence may not always be present but findings can include tissue ischemia, necrosis, extravascular calcification, intimal fibroplasia, thrombosis, and subcutaneous vessel calcium deposits. A wedge biopsy is preferred to a punch biopsy to include the subcutaneous vessels to help with diagnosis. Multiple biopsies may be needed to yield a diagnosis as a single biopsy may be negative for the disease. A risk to consider is the possibility of creating a nonhealing ulcer from biopsy sites. Bone scintigraphy is a noninvasive diagnostic tool that has a sensitivity of 97% in establishing abnormal calcifications. […] Calciphylaxis appears to be multifactorial with unknown pathogenesis and, therefore, no serologic or hematologic confirmatory test is available. However, diagnostic tests that can be included in the initial workup to identify abnormalities include complete blood count, urea and creatinine, corrected calcium, phosphate, calcium-phosphorus index, PTH, coagulation profile, and thrombophilia screen. An elevated calcium-phosphate product (70 mg2/dL2) has a specificity of 95% and sensitivity of 21%. However, normal laboratory values do not rule out the diagnosis of calciphylaxis; 51% of cases in 1 study had calcium-phosphate products less than 50 mg2/dL2.

• #46 Calciphylaxis Prevention and Treatment Strategies- Clinical Advisor

  https://www.clinicaladvisor.com/features/calciphylaxis-prevention-treatment-strategies/

  Diagnosis can be made clinically, although skin biopsy and histology may help in the diagnosis. Histologic evidence may not always be present but findings can include tissue ischemia, necrosis, extravascular calcification, intimal fibroplasia, thrombosis, and subcutaneous vessel calcium deposits. A wedge biopsy is preferred to a punch biopsy to include the subcutaneous vessels to help with diagnosis. Multiple biopsies may be needed to yield a diagnosis as a single biopsy may be negative for the disease. A risk to consider is the possibility of creating a nonhealing ulcer from biopsy sites. Bone scintigraphy is a noninvasive diagnostic tool that has a sensitivity of 97% in establishing abnormal calcifications. […] Calciphylaxis appears to be multifactorial with unknown pathogenesis and, therefore, no serologic or hematologic confirmatory test is available. However, diagnostic tests that can be included in the initial workup to identify abnormalities include complete blood count, urea and creatinine, corrected calcium, phosphate, calcium-phosphorus index, PTH, coagulation profile, and thrombophilia screen. An elevated calcium-phosphate product (70 mg2/dL2) has a specificity of 95% and sensitivity of 21%. However, normal laboratory values do not rule out the diagnosis of calciphylaxis; 51% of cases in 1 study had calcium-phosphate products less than 50 mg2/dL2.

• #47 Calciphylaxis: Pictures, Definition, Symptoms, Treatment, and Outlook

  https://www.healthline.com/health/calciphylaxis

  Calciphylaxis is a kidney complication causing calcium buildup inside fat and skin blood vessels. […] Although a rare condition, if you suspect you have calciphylaxis, do not hesitate to contact your doctor. Early treatment may improve your outlook. […] A doctor might suspect calciphylaxis based on the presence of painful skin lesions. If such physical symptoms are present, they’ll perform a physical examination or skin biopsy both important tools to help confirm calciphylaxis. […] Your doctor might also run several tests to rule out other complications of chronic kidney disease. Some diagnostic tests may include: skin biopsy, blood tests for levels of calcium, phosphorous, alkaline phosphatase, PTH, 25-hydroxyvitamin D, liver function blood tests, kidney function tests, tests to evaluate infections, such as a complete blood count and blood culture tests.

• #48 Calciphylaxis Diagnosis and Management in Primary Care

  https://www.clinicaladvisor.com/features/calciphylaxis-diagnosis-treatment-primary-care/

  Calciphylaxis should be considered in patients presenting with painful nonhealing skin ulcers who have chronic kidney disease, obesity, have been on hemodialysis, and/or are of White race and female sex. Skin changes associated with calciphylaxis have sudden onset and progress rapidly to open ulcerations and then necrosis. A thorough physical examination of the patient, preferably with the torso and lower extremities exposed, can assist the clinician in visualizing the patients lesions and assessing skin integrity. A painful necrotic ulcer covered with black eschar that started as painful ecchymoses is usually the presenting symptom; patients often mistake initial reddening for a bruise and delay seeking medical care. The presence of malodorous, dusky, and/or necrotic lesions should increase suspicion of calciphylaxis. Clinical findings include purple or red net-like areas of ecchymoses that are painful or nonhealing ulcers that are painful. Imaging studies such as radiograph and bone scan may support the diagnosis by allowing for determination of areas of microcalcifications in calciphylaxis. A positive bone scan has a high sensitivity rate of 97% in cases of calciphylaxis. Laboratory tests such as renal function tests, serum calcium, alkaline phosphatase, phosphorous, and vitamin D can evaluate potential risk factors. The gold standard for diagnosing calciphylaxis is skin biopsy. Clinicians with adequate training should perform the skin biopsy to prevent further trauma to skin that is already fragile and difficult to heal. Clinicians must weigh the benefits and risks of biopsy; patients with ESRD and classic presentation of calciphylaxis do not require biopsy. The double-punch technique, where a punch biopsy is followed by a second punch inserted through the center of the first punch, can offer an improved yield on the composition of the skin and underlying structures. Once diagnosis of calciphylaxis is made, referral to a dermatologist is helpful for management of the damaged skin.

• #49 Calciphylaxis: What Causes it and Is it Fatal?

  https://www.webmd.com/skin-problems-and-treatments/what-is-calciphylaxis

  How Is Calciphylaxis Diagnosed? […] If you or a loved one is suffering from these symptoms and is also suffering from kidney injury or kidney failure, your health care practitioner may suspect calciphylaxis. […] A series of tests can be performed to confirm a calciphylaxis diagnosis: […] Skin biopsy. A sample of skin, usually from a lesion or sore, is removed and studied under a microscope. […] Kidney function tests. If you are showing symptoms of calciphylaxis but dont have a history of kidney problems, your healthcare provider may order tests to inspect how efficiently your kidneys are working. […] Blood coagulation tests. These tests reveal if your blood clots and how long it takes. […] General blood tests. A blood sample will be taken and checked for minerals, hormones, and abnormalities. […] Liver function tests. The liver and kidneys are part of your bodys cleaning and filtration system. […] Imaging tests. Some imaging tests like X-rays could be used to diagnose buildup of calcium in the blood vessels.

• #50 Calciphylaxis (Calcific uremic arteriolopathy) – Dermatology Advisor

  https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/calciphylaxis-calcific-uremic-arteriolopathy/

  Autoimmune connective tissue disease, cryoglobulinemia, vasculitides, and hypercoagulable states may mimic calciphylaxis; thus an antinuclear antibody (ANA), antineutrophil cytoplasmic antibody (ANCA), cryoglobulin, cryofibrinogen levels, and hepatitis B/C panels are indicated. […] A deep wedge biopsy is the standard for confirming the diagnosis, as a punch biopsy may be too superficial. […] Biopsies show medial calcification and intimal fibroplasia of small and medium-sized arterioles. […] The value of radiology is limited in the diagnosis of calciphylaxis. […] Mammography is the most sensitive radiologic technique for the detection of arteriolar calcification. […] The differential diagnosis of calciphylaxis includes hematologic abnormalities, hypercoagulable states, infectious etiologies, vasculitides, embolic phenomena, and connective tissue disease.

• #51 Calciphylaxis: What Causes it and Is it Fatal?

  https://www.webmd.com/skin-problems-and-treatments/what-is-calciphylaxis

  How Is Calciphylaxis Diagnosed? […] If you or a loved one is suffering from these symptoms and is also suffering from kidney injury or kidney failure, your health care practitioner may suspect calciphylaxis. […] A series of tests can be performed to confirm a calciphylaxis diagnosis: […] Skin biopsy. A sample of skin, usually from a lesion or sore, is removed and studied under a microscope. […] Kidney function tests. If you are showing symptoms of calciphylaxis but dont have a history of kidney problems, your healthcare provider may order tests to inspect how efficiently your kidneys are working. […] Blood coagulation tests. These tests reveal if your blood clots and how long it takes. […] General blood tests. A blood sample will be taken and checked for minerals, hormones, and abnormalities. […] Liver function tests. The liver and kidneys are part of your bodys cleaning and filtration system. […] Imaging tests. Some imaging tests like X-rays could be used to diagnose buildup of calcium in the blood vessels.

• #52 Calciphylaxis: Pictures, Definition, Symptoms, Treatment, and Outlook

  https://www.healthline.com/health/calciphylaxis

  Calciphylaxis is a kidney complication causing calcium buildup inside fat and skin blood vessels. […] Although a rare condition, if you suspect you have calciphylaxis, do not hesitate to contact your doctor. Early treatment may improve your outlook. […] A doctor might suspect calciphylaxis based on the presence of painful skin lesions. If such physical symptoms are present, they’ll perform a physical examination or skin biopsy both important tools to help confirm calciphylaxis. […] Your doctor might also run several tests to rule out other complications of chronic kidney disease. Some diagnostic tests may include: skin biopsy, blood tests for levels of calcium, phosphorous, alkaline phosphatase, PTH, 25-hydroxyvitamin D, liver function blood tests, kidney function tests, tests to evaluate infections, such as a complete blood count and blood culture tests.

• #53 Calciphylaxis Prevention and Treatment Strategies- Clinical Advisor

  https://www.clinicaladvisor.com/features/calciphylaxis-prevention-treatment-strategies/

  Diagnosis can be made clinically, although skin biopsy and histology may help in the diagnosis. Histologic evidence may not always be present but findings can include tissue ischemia, necrosis, extravascular calcification, intimal fibroplasia, thrombosis, and subcutaneous vessel calcium deposits. A wedge biopsy is preferred to a punch biopsy to include the subcutaneous vessels to help with diagnosis. Multiple biopsies may be needed to yield a diagnosis as a single biopsy may be negative for the disease. A risk to consider is the possibility of creating a nonhealing ulcer from biopsy sites. Bone scintigraphy is a noninvasive diagnostic tool that has a sensitivity of 97% in establishing abnormal calcifications. […] Calciphylaxis appears to be multifactorial with unknown pathogenesis and, therefore, no serologic or hematologic confirmatory test is available. However, diagnostic tests that can be included in the initial workup to identify abnormalities include complete blood count, urea and creatinine, corrected calcium, phosphate, calcium-phosphorus index, PTH, coagulation profile, and thrombophilia screen. An elevated calcium-phosphate product (70 mg2/dL2) has a specificity of 95% and sensitivity of 21%. However, normal laboratory values do not rule out the diagnosis of calciphylaxis; 51% of cases in 1 study had calcium-phosphate products less than 50 mg2/dL2.

• #54 FF #325 Uremic Calciphylaxis | Palliative Care Network of Wisconsin

  https://www.mypcnow.org/fast-fact/uremic-calciphylaxis/

  Calciphylaxis is a poorly understood disorder in which calcification of small blood vessels causes painful ischemic skin and visceral lesions most often in patients with end-stage renal disease (ESRD). […] Calciphylaxis is a clinical diagnosis. Laboratory findings are non-specific. In certain circumstances, a dermatology consult and/or skin biopsy may be needed. However, skin biopsy is usually deferred due to risk of pain, a false negative result, and poor wound healing. […] Imaging studies can support the diagnosis by identifying calcification, but they do not confirm a diagnosis and may lead to unnecessary discomfort.

• #55 Calciphylaxis Workup: Laboratory Studies, Imaging Studies, Procedures

  https://emedicine.medscape.com/article/1095481-workup

  In the workup for calciphylaxis (also referred to as calcific uremic arteriolopathy), the following laboratory measurements may be considered: […] Plain radiography uniformly demonstrates an arborization of vascular calcification within the dermis and the subcutaneous tissue. […] A study by Shmidt et al showed that patients with calciphylaxis had more vascular calcifications, more small-vessel calcifications, and a netlike pattern of calcifications. This netlike pattern, when present, was strongly associated with the presence of calciphylaxis. […] Bone scintigraphy may be used as a noninvasive diagnostic tool because the bone matrix protein osteopontin has been demonstrated in calciphylaxis lesions. […] Biopsy specimens typically demonstrate calcification within the media and intima of small and medium-sized arterioles with extensive intimal hyperplasia and fibrosis, but this also may be seen in uninvolved skin in patients with chronic kidney disease (CKD; or chronic renal failure) or atherosclerosis. […] The decision to perform a biopsy on a nonulcerated lesion should not be made lightly, because it could result in a nonhealing wound. […] Although calciphylaxis lesions have a clinical appearance suggestive of avascular necrosis (AVN), the tissue often bleeds freely during surgery.

• #56 Calciphylaxis: Causes, Symptoms, and Management — DermNet

  https://dermnetnz.org/topics/calciphylaxis

  Bone scintigraphy using technetium Tc 99m bisphosphonates in patients with calciphylaxis shows increased radiotracer uptake in soft tissues throughout the body and is specifically enhanced in indurated plaques affected by calciphylaxis (but is absent in ulcers due to reduced blood flow at sites of tissue necrosis).

• #57 Calciphylaxis Diagnosis and Management in Primary Care

  https://www.clinicaladvisor.com/features/calciphylaxis-diagnosis-treatment-primary-care/

  Calciphylaxis should be considered in patients presenting with painful nonhealing skin ulcers who have chronic kidney disease, obesity, have been on hemodialysis, and/or are of White race and female sex. Skin changes associated with calciphylaxis have sudden onset and progress rapidly to open ulcerations and then necrosis. A thorough physical examination of the patient, preferably with the torso and lower extremities exposed, can assist the clinician in visualizing the patients lesions and assessing skin integrity. A painful necrotic ulcer covered with black eschar that started as painful ecchymoses is usually the presenting symptom; patients often mistake initial reddening for a bruise and delay seeking medical care. The presence of malodorous, dusky, and/or necrotic lesions should increase suspicion of calciphylaxis. Clinical findings include purple or red net-like areas of ecchymoses that are painful or nonhealing ulcers that are painful. Imaging studies such as radiograph and bone scan may support the diagnosis by allowing for determination of areas of microcalcifications in calciphylaxis. A positive bone scan has a high sensitivity rate of 97% in cases of calciphylaxis. Laboratory tests such as renal function tests, serum calcium, alkaline phosphatase, phosphorous, and vitamin D can evaluate potential risk factors. The gold standard for diagnosing calciphylaxis is skin biopsy. Clinicians with adequate training should perform the skin biopsy to prevent further trauma to skin that is already fragile and difficult to heal. Clinicians must weigh the benefits and risks of biopsy; patients with ESRD and classic presentation of calciphylaxis do not require biopsy. The double-punch technique, where a punch biopsy is followed by a second punch inserted through the center of the first punch, can offer an improved yield on the composition of the skin and underlying structures. Once diagnosis of calciphylaxis is made, referral to a dermatologist is helpful for management of the damaged skin.

• #58 Role of imaging in calciphylaxis diagnosis | Eurorad

  https://www.eurorad.org/case/16949

  0 Role of imaging in calciphylaxis diagnosis […] The role of radiology in the diagnosis is not well established. Different imaging modalities can detect extraosseous calcium deposition. […] Mammography can detect calcification in arteries as small as 0,13mm in calibre and provides a better resolution than conventional X-Ray or CT scan. […] Calcification of small arterioles in a netlike pattern have been described as the key imaging feature and it is highly suggestive of the diagnosis in symptomatic patients […] Nuclear medicine techniques such as bone scintigraphy using Tc99 have a high sensitivity in detection of extraosseous calcifications and can assess both extent as well as presence of active disease. […] Final diagnosis requires a skin biopsy showing characteristic histopathological features including medial calcification, intimal proliferation and microthrombi in small arteries. Extravascular interstitial calcium deposition, especially in perieccrine location, as well as resultant ischemic necrosis and inflammatory changes also help establish the diagnosis.

• #59 Calciphylaxis (Calcific uremic arteriolopathy) – Dermatology Advisor

  https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/calciphylaxis-calcific-uremic-arteriolopathy/

  Autoimmune connective tissue disease, cryoglobulinemia, vasculitides, and hypercoagulable states may mimic calciphylaxis; thus an antinuclear antibody (ANA), antineutrophil cytoplasmic antibody (ANCA), cryoglobulin, cryofibrinogen levels, and hepatitis B/C panels are indicated. […] A deep wedge biopsy is the standard for confirming the diagnosis, as a punch biopsy may be too superficial. […] Biopsies show medial calcification and intimal fibroplasia of small and medium-sized arterioles. […] The value of radiology is limited in the diagnosis of calciphylaxis. […] Mammography is the most sensitive radiologic technique for the detection of arteriolar calcification. […] The differential diagnosis of calciphylaxis includes hematologic abnormalities, hypercoagulable states, infectious etiologies, vasculitides, embolic phenomena, and connective tissue disease.

• #60 Radiomics-based method for diagnosis of calciphylaxis in patients with chronic kidney disease using computed tomography – Yu – Quantitative Imaging in Medicine and Surgery

  https://qims.amegroups.org/article/view/73274/html

  Calciphylaxis diagnosis is challenging, for which skin biopsy is a gold standard. Nonetheless, it increases the risk of ulceration, bleeding, and necrosis owing to deep incisions. A noninvasive tool for diagnosing calciphylaxis lesions is beneficial for patients with skin lesions and CKD. […] This study aimed to develop radiomic methods using CT as a noninvasive method for calciphylaxis diagnosis. […] In this research, it primarily developed a radiomic method for noninvasive detection of calciphylaxis in patients with CKD. Through this method, calciphylaxis can be detected when invasive procedures are not feasible. […] The model, based on 8 features including 4 first-order features and 4 textural features, showed the highest AUC on the validation data set. […] The exploration of diagnostic models has confirmed the possibility of a non-invasive diagnosis method that might provide clinicians with an alternative when histopathological examination is impossible and thereby reducing the incidence of complications due to invasive operations, especially at the skin lesions.

• #61 Radiomics-based method for diagnosis of calciphylaxis in patients with chronic kidney disease using computed tomography – Yu – Quantitative Imaging in Medicine and Surgery

  https://qims.amegroups.org/article/view/73274/html

  To the best of our knowledge, this research is the first to develop a radiomics method in calciphylaxis diagnosis. CT radiomics features hidden within skin lesions were extracted and the radiomic model demonstrated preliminary feasibility as a noninvasive technique for calciphylaxis diagnosis in patients with CKD when invasive procedures are not available.

• #62 A Possible Role For POCUS in the Diagnosis of Calciphylaxis — BROWN EMERGENCY MEDICINE BLOG

  http://brownemblog.com/blogposts/2023/1/16/a-possible-role-for-pocus-in-the-diagnosis-of-calciphylaxis

  Calciphylaxis is a life-threatening vasculopathy that results from the deposition of calcium in the arteriolar vasculature of the deep dermis and subcutaneous adipose tissue, which causes occlusion of the affected blood vessels and overlying tissues and skin. […] Despite the dramatic signs and symptoms associated with calciphylaxis, making this diagnosis solely on clinical grounds can be challenging: retiform purpura is a cutaneous manifestation that commands a wide differential (including cryoglobulinemia, polyarteritis nodosa and other vasculitides, heparin-induced thrombocytopenia, to name a few) and therefore these lesions are not specific to calciphylaxis. […] Recent reports have suggested a possible role for point-of-care ultrasound (POCUS) in the diagnosis of calciphylaxis. […] One report presented a case of penile calciphylaxis that was successfully identified through POCUS after two negative punch biopsies were obtained, suggesting that POCUS may be an additional tool to confirm this condition in the setting of non-diagnostic punch biopsies.

• #63 A Possible Role For POCUS in the Diagnosis of Calciphylaxis — BROWN EMERGENCY MEDICINE BLOG

  http://brownemblog.com/blogposts/2023/1/16/a-possible-role-for-pocus-in-the-diagnosis-of-calciphylaxis

  While randomized-controlled studies are needed to ascertain the utility of POCUS in the diagnosis of calciphylaxis and further characterization of the sonographic features that are sensitive and specific to this inflammatory dermatosis are needed, early reports suggest that this non-invasive imaging modality may be of diagnostic aid and therefore facilitate the early initiation of treatment.

• #64

  https://journals.lww.com/aswcjournal/fulltext/2019/05000/calciphylaxis__diagnosis,_pathogenesis,_and.3.aspx

  To provide information on the pathogenesis, clinical features, diagnosis, and treatment of calciphylaxis. […] Explain the diagnosis and management of a patient with calciphylaxis. […] Diagnosis can be made on clinical grounds alone when a patient with ESRD presents with indurated tender plaques or ulcers on the abdomen and/or legs. […] Skin biopsy may be necessary to differentiate calciphylaxis from its mimics (eg, pyoderma gangrenosum, Martorell hypertensive ischemic leg ulcer, cholesterol emboli, etc; Table 2), especially when calciphylaxis presents in early stages with clinically nondescript lesions such as a small papule and erosion. […] In contrast, skin biopsy is not always indicated in uremic calciphylaxis because the likelihood of calciphylaxis is much higher when an ESRD patient presents with characteristic skin lesions, as compared with patients with preserved renal function.

• #65 Calciphylaxis or Calcific Uremic Arteriolopathy: Diagnosis not to be Overlooked in Hemodialysis Patients

  https://www.heraldopenaccess.us/openaccess/calciphylaxis-or-calcific-uremic-arteriolopathy-diagnosis-not-to-be-overlooked-in-hemodialysis-patients

  Calciphylaxis or calcific uremic arteriolopathy is a rare and severe condition due to its painful ischemic necrosis cutaneous and sometimes systemic lesions, whose functional and vital prognosis is reserved due to infectious complications. […] Its diagnosis is rarely mentioned, leading the clinician to multiple differential diagnoses. […] The diagnosis must be urgent. […] The clinic is dominated by lesions of livedo, ulcers or necrotic plaques of locations often distal as in our patient who had lesions in the leg and heel, or proximal or in the fatty areas (trunk, abdomen). […] It can pose a problem of differential diagnosis with cholesterol embolism, anti-phospholipid syndrome, anti-vitamin k necrosis, bullous dermatitis, ocher ulcer of venous insufficiency and nephrogenic systemic fibrosis.

• #66 Calciphylaxis Prevention and Treatment Strategies- Clinical Advisor

  https://www.clinicaladvisor.com/features/calciphylaxis-prevention-treatment-strategies/

  Differential diagnosis of the cutaneous lesions seen in calciphylaxis includes venous stasis ulcers, pyoderma gangrenosum, vasculitis, brown recluse spider bite, and necrotizing fasciitis. Other conditions to consider are disseminated intravascular coagulation, clotting disorders, antiphospholipid syndrome, infections, cryoprecipitate disorders, cholesterol emboli, atheroemboli, marantic endocarditis, warfarin-induced skin necrosis, and myxoma.

• #67

  https://journals.lww.com/aswcjournal/fulltext/2019/05000/calciphylaxis__diagnosis,_pathogenesis,_and.3.aspx

  To provide information on the pathogenesis, clinical features, diagnosis, and treatment of calciphylaxis. […] Explain the diagnosis and management of a patient with calciphylaxis. […] Diagnosis can be made on clinical grounds alone when a patient with ESRD presents with indurated tender plaques or ulcers on the abdomen and/or legs. […] Skin biopsy may be necessary to differentiate calciphylaxis from its mimics (eg, pyoderma gangrenosum, Martorell hypertensive ischemic leg ulcer, cholesterol emboli, etc; Table 2), especially when calciphylaxis presents in early stages with clinically nondescript lesions such as a small papule and erosion. […] In contrast, skin biopsy is not always indicated in uremic calciphylaxis because the likelihood of calciphylaxis is much higher when an ESRD patient presents with characteristic skin lesions, as compared with patients with preserved renal function.

• #68

  https://journals.lww.com/aswcjournal/fulltext/2019/05000/calciphylaxis__diagnosis,_pathogenesis,_and.3.aspx

  To provide information on the pathogenesis, clinical features, diagnosis, and treatment of calciphylaxis. […] Explain the diagnosis and management of a patient with calciphylaxis. […] Diagnosis can be made on clinical grounds alone when a patient with ESRD presents with indurated tender plaques or ulcers on the abdomen and/or legs. […] Skin biopsy may be necessary to differentiate calciphylaxis from its mimics (eg, pyoderma gangrenosum, Martorell hypertensive ischemic leg ulcer, cholesterol emboli, etc; Table 2), especially when calciphylaxis presents in early stages with clinically nondescript lesions such as a small papule and erosion. […] In contrast, skin biopsy is not always indicated in uremic calciphylaxis because the likelihood of calciphylaxis is much higher when an ESRD patient presents with characteristic skin lesions, as compared with patients with preserved renal function.

• #69

  https://journals.lww.com/aswcjournal/fulltext/2019/05000/calciphylaxis__diagnosis,_pathogenesis,_and.3.aspx

  To provide information on the pathogenesis, clinical features, diagnosis, and treatment of calciphylaxis. […] Explain the diagnosis and management of a patient with calciphylaxis. […] Diagnosis can be made on clinical grounds alone when a patient with ESRD presents with indurated tender plaques or ulcers on the abdomen and/or legs. […] Skin biopsy may be necessary to differentiate calciphylaxis from its mimics (eg, pyoderma gangrenosum, Martorell hypertensive ischemic leg ulcer, cholesterol emboli, etc; Table 2), especially when calciphylaxis presents in early stages with clinically nondescript lesions such as a small papule and erosion. […] In contrast, skin biopsy is not always indicated in uremic calciphylaxis because the likelihood of calciphylaxis is much higher when an ESRD patient presents with characteristic skin lesions, as compared with patients with preserved renal function.

• #70 Calciphylaxis or Calcific Uremic Arteriolopathy: Diagnosis not to be Overlooked in Hemodialysis Patients

  https://www.heraldopenaccess.us/openaccess/calciphylaxis-or-calcific-uremic-arteriolopathy-diagnosis-not-to-be-overlooked-in-hemodialysis-patients

  Calciphylaxis or calcific uremic arteriolopathy is a rare and severe condition due to its painful ischemic necrosis cutaneous and sometimes systemic lesions, whose functional and vital prognosis is reserved due to infectious complications. […] Its diagnosis is rarely mentioned, leading the clinician to multiple differential diagnoses. […] The diagnosis must be urgent. […] The clinic is dominated by lesions of livedo, ulcers or necrotic plaques of locations often distal as in our patient who had lesions in the leg and heel, or proximal or in the fatty areas (trunk, abdomen). […] It can pose a problem of differential diagnosis with cholesterol embolism, anti-phospholipid syndrome, anti-vitamin k necrosis, bullous dermatitis, ocher ulcer of venous insufficiency and nephrogenic systemic fibrosis.

• #71 Calciphylaxis: Causes, Symptoms, and Management — DermNet

  https://dermnetnz.org/topics/calciphylaxis

  How is calciphylaxis diagnosed? A deep wedge skin biopsy may be necessary to diagnose calciphylaxis, as a similar appearance can be seen in other conditions such as necrotising fasciitis, cryoglobulinaemia, antiphospholipid syndrome, coumarin necrosis and vasculitis. Multiple biopsies may be necessary, with a risk of propagating calciphylaxis. The pathologist looks for calcium deposited within scarred and blocked blood vessels in the subcutaneous tissue. Perieccrine calcium deposition may be noted when vascular calcification is absent but may be subtle. There may also be inflammation of the fat (panniculitis). […] X-rays of the affected limb may demonstrate vascular calcification within the skin; however, this may also be seen in healthy patients with renal disease that are not affected by calciphylaxis.

• #72 Calciphylaxis or Calcific Uremic Arteriolopathy: Diagnosis not to be Overlooked in Hemodialysis Patients

  https://www.heraldopenaccess.us/openaccess/calciphylaxis-or-calcific-uremic-arteriolopathy-diagnosis-not-to-be-overlooked-in-hemodialysis-patients

  Calciphylaxis or calcific uremic arteriolopathy is a rare and severe condition due to its painful ischemic necrosis cutaneous and sometimes systemic lesions, whose functional and vital prognosis is reserved due to infectious complications. […] Its diagnosis is rarely mentioned, leading the clinician to multiple differential diagnoses. […] The diagnosis must be urgent. […] The clinic is dominated by lesions of livedo, ulcers or necrotic plaques of locations often distal as in our patient who had lesions in the leg and heel, or proximal or in the fatty areas (trunk, abdomen). […] It can pose a problem of differential diagnosis with cholesterol embolism, anti-phospholipid syndrome, anti-vitamin k necrosis, bullous dermatitis, ocher ulcer of venous insufficiency and nephrogenic systemic fibrosis.

• #73 Calciphylaxis: Causes, Symptoms, and Management — DermNet

  https://dermnetnz.org/topics/calciphylaxis

  How is calciphylaxis diagnosed? A deep wedge skin biopsy may be necessary to diagnose calciphylaxis, as a similar appearance can be seen in other conditions such as necrotising fasciitis, cryoglobulinaemia, antiphospholipid syndrome, coumarin necrosis and vasculitis. Multiple biopsies may be necessary, with a risk of propagating calciphylaxis. The pathologist looks for calcium deposited within scarred and blocked blood vessels in the subcutaneous tissue. Perieccrine calcium deposition may be noted when vascular calcification is absent but may be subtle. There may also be inflammation of the fat (panniculitis). […] X-rays of the affected limb may demonstrate vascular calcification within the skin; however, this may also be seen in healthy patients with renal disease that are not affected by calciphylaxis.

• #74 Calciphylaxis: Causes, Symptoms, and Management — DermNet

  https://dermnetnz.org/topics/calciphylaxis

  How is calciphylaxis diagnosed? A deep wedge skin biopsy may be necessary to diagnose calciphylaxis, as a similar appearance can be seen in other conditions such as necrotising fasciitis, cryoglobulinaemia, antiphospholipid syndrome, coumarin necrosis and vasculitis. Multiple biopsies may be necessary, with a risk of propagating calciphylaxis. The pathologist looks for calcium deposited within scarred and blocked blood vessels in the subcutaneous tissue. Perieccrine calcium deposition may be noted when vascular calcification is absent but may be subtle. There may also be inflammation of the fat (panniculitis). […] X-rays of the affected limb may demonstrate vascular calcification within the skin; however, this may also be seen in healthy patients with renal disease that are not affected by calciphylaxis.

• #75 Calciphylaxis Prevention and Treatment Strategies- Clinical Advisor

  https://www.clinicaladvisor.com/features/calciphylaxis-prevention-treatment-strategies/

  Differential diagnosis of the cutaneous lesions seen in calciphylaxis includes venous stasis ulcers, pyoderma gangrenosum, vasculitis, brown recluse spider bite, and necrotizing fasciitis. Other conditions to consider are disseminated intravascular coagulation, clotting disorders, antiphospholipid syndrome, infections, cryoprecipitate disorders, cholesterol emboli, atheroemboli, marantic endocarditis, warfarin-induced skin necrosis, and myxoma.

• #76 Calciphylaxis Prevention and Treatment Strategies- Clinical Advisor

  https://www.clinicaladvisor.com/features/calciphylaxis-prevention-treatment-strategies/

  Differential diagnosis of the cutaneous lesions seen in calciphylaxis includes venous stasis ulcers, pyoderma gangrenosum, vasculitis, brown recluse spider bite, and necrotizing fasciitis. Other conditions to consider are disseminated intravascular coagulation, clotting disorders, antiphospholipid syndrome, infections, cryoprecipitate disorders, cholesterol emboli, atheroemboli, marantic endocarditis, warfarin-induced skin necrosis, and myxoma.

• #77 Calciphylaxis Prevention and Treatment Strategies- Clinical Advisor

  https://www.clinicaladvisor.com/features/calciphylaxis-prevention-treatment-strategies/

  Differential diagnosis of the cutaneous lesions seen in calciphylaxis includes venous stasis ulcers, pyoderma gangrenosum, vasculitis, brown recluse spider bite, and necrotizing fasciitis. Other conditions to consider are disseminated intravascular coagulation, clotting disorders, antiphospholipid syndrome, infections, cryoprecipitate disorders, cholesterol emboli, atheroemboli, marantic endocarditis, warfarin-induced skin necrosis, and myxoma.

• #78 Calciphylaxis and its diagnosis: A review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6820424/

  Calciphylaxis also known as Calcific uremic arteriolopathy (CUA), is a rare fatal complication usually associated with end-stage renal disease (ESRD). […] Skin biopsy and radiographic features are helpful in the diagnosis of calciphylaxis, but negative results do not necessarily exclude the diagnosis. This article highlights steps undertaking in the diagnosis of calciphylaxis. […] Intense pain associated with palpation of firm calcified subcutaneous tissue and cutaneous lesions is suggestive of calciphylaxis in dialysis patients and in patients with other risk factors for the disorder. […] Various steps that are undertaken for accurate diagnosis of the disease are as follows. […] The clinical examination of a patient with calciphylaxis involves two important goals: to evaluate the presence of any etiological factor and to rule out any potential disorders that may mimic the physical examination findings.

• #79 Calciphylaxis Diagnosis and Management in Primary Care

  https://www.clinicaladvisor.com/features/calciphylaxis-diagnosis-treatment-primary-care/

  Calciphylaxis should be considered in patients presenting with painful nonhealing skin ulcers who have chronic kidney disease, obesity, have been on hemodialysis, and/or are of White race and female sex. Skin changes associated with calciphylaxis have sudden onset and progress rapidly to open ulcerations and then necrosis. A thorough physical examination of the patient, preferably with the torso and lower extremities exposed, can assist the clinician in visualizing the patients lesions and assessing skin integrity. A painful necrotic ulcer covered with black eschar that started as painful ecchymoses is usually the presenting symptom; patients often mistake initial reddening for a bruise and delay seeking medical care. The presence of malodorous, dusky, and/or necrotic lesions should increase suspicion of calciphylaxis. Clinical findings include purple or red net-like areas of ecchymoses that are painful or nonhealing ulcers that are painful. Imaging studies such as radiograph and bone scan may support the diagnosis by allowing for determination of areas of microcalcifications in calciphylaxis. A positive bone scan has a high sensitivity rate of 97% in cases of calciphylaxis. Laboratory tests such as renal function tests, serum calcium, alkaline phosphatase, phosphorous, and vitamin D can evaluate potential risk factors. The gold standard for diagnosing calciphylaxis is skin biopsy. Clinicians with adequate training should perform the skin biopsy to prevent further trauma to skin that is already fragile and difficult to heal. Clinicians must weigh the benefits and risks of biopsy; patients with ESRD and classic presentation of calciphylaxis do not require biopsy. The double-punch technique, where a punch biopsy is followed by a second punch inserted through the center of the first punch, can offer an improved yield on the composition of the skin and underlying structures. Once diagnosis of calciphylaxis is made, referral to a dermatologist is helpful for management of the damaged skin.

• #80 Calciphylaxis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/calciphylaxis/diagnosis-treatment/drc-20370562

  Diagnosis involves finding out if calciphylaxis is the cause of your symptoms. Your healthcare professional reviews your health history, asks about your symptoms and gives you a physical exam. […] You also may need tests such as: […] Skin biopsy. During this procedure, your healthcare professional removes a small tissue sample from an area of affected skin. Then, a lab checks the sample. […] Blood tests. A lab can measure various substances in your blood. These include creatinine, calcium, phosphorus, parathyroid hormone and vitamin D. The results help your healthcare team check how well your kidneys are working. […] Imaging tests. These can be useful if biopsy results aren’t clear or if a biopsy can’t be done. X-rays may show calcium buildups in the blood vessels. These buildups are common in calciphylaxis and in other advanced kidney diseases.

• #81 Calciphylaxis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/calciphylaxis/diagnosis-treatment/drc-20370562

  Diagnosis involves finding out if calciphylaxis is the cause of your symptoms. Your healthcare professional reviews your health history, asks about your symptoms and gives you a physical exam. […] You also may need tests such as: […] Skin biopsy. During this procedure, your healthcare professional removes a small tissue sample from an area of affected skin. Then, a lab checks the sample. […] Blood tests. A lab can measure various substances in your blood. These include creatinine, calcium, phosphorus, parathyroid hormone and vitamin D. The results help your healthcare team check how well your kidneys are working. […] Imaging tests. These can be useful if biopsy results aren’t clear or if a biopsy can’t be done. X-rays may show calcium buildups in the blood vessels. These buildups are common in calciphylaxis and in other advanced kidney diseases.

• #82 Calciphylaxis Diagnosis and Management in Primary Care

  https://www.clinicaladvisor.com/features/calciphylaxis-diagnosis-treatment-primary-care/

  Calciphylaxis should be considered in patients presenting with painful nonhealing skin ulcers who have chronic kidney disease, obesity, have been on hemodialysis, and/or are of White race and female sex. Skin changes associated with calciphylaxis have sudden onset and progress rapidly to open ulcerations and then necrosis. A thorough physical examination of the patient, preferably with the torso and lower extremities exposed, can assist the clinician in visualizing the patients lesions and assessing skin integrity. A painful necrotic ulcer covered with black eschar that started as painful ecchymoses is usually the presenting symptom; patients often mistake initial reddening for a bruise and delay seeking medical care. The presence of malodorous, dusky, and/or necrotic lesions should increase suspicion of calciphylaxis. Clinical findings include purple or red net-like areas of ecchymoses that are painful or nonhealing ulcers that are painful. Imaging studies such as radiograph and bone scan may support the diagnosis by allowing for determination of areas of microcalcifications in calciphylaxis. A positive bone scan has a high sensitivity rate of 97% in cases of calciphylaxis. Laboratory tests such as renal function tests, serum calcium, alkaline phosphatase, phosphorous, and vitamin D can evaluate potential risk factors. The gold standard for diagnosing calciphylaxis is skin biopsy. Clinicians with adequate training should perform the skin biopsy to prevent further trauma to skin that is already fragile and difficult to heal. Clinicians must weigh the benefits and risks of biopsy; patients with ESRD and classic presentation of calciphylaxis do not require biopsy. The double-punch technique, where a punch biopsy is followed by a second punch inserted through the center of the first punch, can offer an improved yield on the composition of the skin and underlying structures. Once diagnosis of calciphylaxis is made, referral to a dermatologist is helpful for management of the damaged skin.

• #83 Calciphylaxis Diagnosis and Management in Primary Care

  https://www.clinicaladvisor.com/features/calciphylaxis-diagnosis-treatment-primary-care/

  Calciphylaxis should be considered in patients presenting with painful nonhealing skin ulcers who have chronic kidney disease, obesity, have been on hemodialysis, and/or are of White race and female sex. Skin changes associated with calciphylaxis have sudden onset and progress rapidly to open ulcerations and then necrosis. A thorough physical examination of the patient, preferably with the torso and lower extremities exposed, can assist the clinician in visualizing the patients lesions and assessing skin integrity. A painful necrotic ulcer covered with black eschar that started as painful ecchymoses is usually the presenting symptom; patients often mistake initial reddening for a bruise and delay seeking medical care. The presence of malodorous, dusky, and/or necrotic lesions should increase suspicion of calciphylaxis. Clinical findings include purple or red net-like areas of ecchymoses that are painful or nonhealing ulcers that are painful. Imaging studies such as radiograph and bone scan may support the diagnosis by allowing for determination of areas of microcalcifications in calciphylaxis. A positive bone scan has a high sensitivity rate of 97% in cases of calciphylaxis. Laboratory tests such as renal function tests, serum calcium, alkaline phosphatase, phosphorous, and vitamin D can evaluate potential risk factors. The gold standard for diagnosing calciphylaxis is skin biopsy. Clinicians with adequate training should perform the skin biopsy to prevent further trauma to skin that is already fragile and difficult to heal. Clinicians must weigh the benefits and risks of biopsy; patients with ESRD and classic presentation of calciphylaxis do not require biopsy. The double-punch technique, where a punch biopsy is followed by a second punch inserted through the center of the first punch, can offer an improved yield on the composition of the skin and underlying structures. Once diagnosis of calciphylaxis is made, referral to a dermatologist is helpful for management of the damaged skin.

• #84 Advanced-stage calciphylaxis: Think before you punch | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/83/8/562

  In the advanced stages, the diagnosis of calciphylaxis is clinically more evident, and the differential diagnosis usually narrows. […] Well-demarcated, necrotic, indurated lesions that are bilateral in a patient with end-stage renal disease without shock makes the diagnosis very likely. […] Biopsy for histologic confirmation of calciphylaxis can increase the risk of infection and sepsis. […] Since extensive necrosis predisposes to a negative biopsy, a high clinical suspicion should drive early treatment of calciphylaxis. […] Noninvasive imaging studies such as plain radiography and bone scintigraphy can aid the diagnosis by detecting moderate to severe soft-tissue vascular calcification in these areas.

• #85 Calciphylaxis Diagnosis and Management in Primary Care

  https://www.clinicaladvisor.com/features/calciphylaxis-diagnosis-treatment-primary-care/

  Calciphylaxis should be considered in patients presenting with painful nonhealing skin ulcers who have chronic kidney disease, obesity, have been on hemodialysis, and/or are of White race and female sex. Skin changes associated with calciphylaxis have sudden onset and progress rapidly to open ulcerations and then necrosis. A thorough physical examination of the patient, preferably with the torso and lower extremities exposed, can assist the clinician in visualizing the patients lesions and assessing skin integrity. A painful necrotic ulcer covered with black eschar that started as painful ecchymoses is usually the presenting symptom; patients often mistake initial reddening for a bruise and delay seeking medical care. The presence of malodorous, dusky, and/or necrotic lesions should increase suspicion of calciphylaxis. Clinical findings include purple or red net-like areas of ecchymoses that are painful or nonhealing ulcers that are painful. Imaging studies such as radiograph and bone scan may support the diagnosis by allowing for determination of areas of microcalcifications in calciphylaxis. A positive bone scan has a high sensitivity rate of 97% in cases of calciphylaxis. Laboratory tests such as renal function tests, serum calcium, alkaline phosphatase, phosphorous, and vitamin D can evaluate potential risk factors. The gold standard for diagnosing calciphylaxis is skin biopsy. Clinicians with adequate training should perform the skin biopsy to prevent further trauma to skin that is already fragile and difficult to heal. Clinicians must weigh the benefits and risks of biopsy; patients with ESRD and classic presentation of calciphylaxis do not require biopsy. The double-punch technique, where a punch biopsy is followed by a second punch inserted through the center of the first punch, can offer an improved yield on the composition of the skin and underlying structures. Once diagnosis of calciphylaxis is made, referral to a dermatologist is helpful for management of the damaged skin.

• #86 Calciphylaxis Program

  https://www.massgeneral.org/medicine/nephrology/treatments-and-services/calciphylaxis

  Calciphylaxis is a rare and serious disorder characterized by reduction in blood flow to skin tissue due to a buildup of calcium in the walls of blood vessels. Calciphylaxis predominantly affects individuals who have advanced kidney disease. The disorder, however, is not restricted to individuals with kidney disease. Calciphylaxis typically manifests with painful skin abnormalities like skin discoloration, a nodule or an ulcer. Unfortunately, individuals with calciphylaxis are at a high risk for skin infection, hospitalization and death. […] At Mass General, we provide a comprehensive interdisciplinary care encompassing diagnosis, treatment and prevention of calciphylaxis. Our personalized care approach starts with establishing the diagnosis of calciphylaxis with an utmost certainty and a thorough evaluation of risk factors.

• #87 Calciphylaxis: Approach to Diagnosis and Management – Making the Rounds

  https://internalmedicineiowa.org/2020/01/13/calciphylaxis-approach-to-diagnosis-and-management/

  Calciphylaxis is a rare disorder of poor prognosis that can lead to intense, painful lesions involving the skin and subcutaneous tissue. […] The diagnosis of calciphylaxis is complicated by the absence of a gold standard marker of disease such as a clear histopathological finding. […] Late diagnosis and advanced lesions can significantly shorten life expectancy. […] Future research is required to establish clear causal pathways and improve on the treatment options currently available to patients.

• #88

  https://scholars.duke.edu/publication/1590446

  Calciphylaxis is an uncommon but devastating disorder characterized by vascular calcification and subsequent cutaneous tissue necrosis. This results in exquisitely painful and slow healing wounds that portend exceptionally high morbidity and mortality. The diagnosis of this condition can be complicated because there are no conclusive serologic, radiographic or visual signs that this disease is manifesting. The differential of tissue necrosis is broad, and identifying calciphylaxis requires an adroit understanding of the risk factors and physical signs that should raise suspicion of this condition. […] Reviews on this subject are uncommon and lack directed commentary from disease experts on the best diagnostic approach for patients suffering from this disease. The goal of this article is to update practicing dermatologists on the current standard of care for calciphylaxis.

• #89 Calciphylaxis: Approach to Diagnosis and Management – Making the Rounds

  https://internalmedicineiowa.org/2020/01/13/calciphylaxis-approach-to-diagnosis-and-management/

  Calciphylaxis is a rare disorder of poor prognosis that can lead to intense, painful lesions involving the skin and subcutaneous tissue. […] The diagnosis of calciphylaxis is complicated by the absence of a gold standard marker of disease such as a clear histopathological finding. […] Late diagnosis and advanced lesions can significantly shorten life expectancy. […] Future research is required to establish clear causal pathways and improve on the treatment options currently available to patients.

• #90 Calciphylaxis

  https://mobile.fpnotebook.com/Renal/Derm/Clcphylxs.htm

  Calciphylaxis (Calcific Uremic Arteriolopathy) […] Rare, often lethal complication of Hemodialysis in End Stage Renal Disease. […] Characterized by cutaneous arteriolar calcification, thrombosis and skin necrosis. […] Calciphylaxis is often a clinical diagnosis that does not require skin biopsy. […] Under-recognized condition outside of Hemodialysis centers (missed diagnosis is not uncommon). […] Very high morbidity and mortality. […] Skin ischemia may progress to skin necrosis. […] One year survival is 50%. […] Mortality typically due to Sepsis. […] […] […] Skin Biopsy […] Precautions […] Skin biopsy sites may heal poorly and risk infection. […] Findings […] Tunica intima layer fibrosis. […] Small artery and arteriole calcifications (esp. capillary calcification within adipose tissue).

• #91 FF #325 Uremic Calciphylaxis | Palliative Care Network of Wisconsin

  https://www.mypcnow.org/fast-fact/uremic-calciphylaxis/

  Calciphylaxis is a poorly understood disorder in which calcification of small blood vessels causes painful ischemic skin and visceral lesions most often in patients with end-stage renal disease (ESRD). […] Calciphylaxis is a clinical diagnosis. Laboratory findings are non-specific. In certain circumstances, a dermatology consult and/or skin biopsy may be needed. However, skin biopsy is usually deferred due to risk of pain, a false negative result, and poor wound healing. […] Imaging studies can support the diagnosis by identifying calcification, but they do not confirm a diagnosis and may lead to unnecessary discomfort.

• #92

  https://link.springer.com/article/10.1007/s12325-020-01504-w

  Calciphylaxis is a deadly, painful disease with a 1-year mortality of up to 50%. […] Diagnosis can be difficult, and the condition can clinically appear similar to other dermatological diseases, especially in non-uremic patients. Currently, skin biopsy with histological analysis is the most reliable method to help diagnose the condition. […] The diagnosis of calciphylaxis stems from the insidious nature of its progression; the early stages of development, including the medial calcification and subsequent adverse tissue effects, occur alongside severe pain in the afflicted regions in the majority of examined cases. […] Even once skin lesions present in a patient, the diagnosis can be difficult to make. […] Currently, the best diagnostic technique in the setting of an unclear clinical presentation is a skin biopsy and histological examination.

• #93 Calciphylaxis Diagnosis and Management in Primary Care

  https://www.renalandurologynews.com/features/calciphylaxis-diagnosis-treatment-primary-care-2/

  Calciphylaxis should be considered in patients presenting with painful nonhealing skin ulcers who have chronic kidney disease, obesity, have been on hemodialysis, and/or are of White race and female sex. Skin changes associated with calciphylaxis have sudden onset and progress rapidly to open ulcerations and then necrosis. A thorough physical examination of the patient, preferably with the torso and lower extremities exposed, can assist the clinician in visualizing the patients lesions and assessing skin integrity. A painful necrotic ulcer covered with black eschar that started as painful ecchymoses is usually the presenting symptom; patients often mistake initial reddening for a bruise and delay seeking medical care. The presence of malodorous, dusky, and/or necrotic lesions should increase suspicion of calciphylaxis. Clinical findings include purple or red net-like areas of ecchymoses that are painful or nonhealing ulcers that are painful. Imaging studies such as radiograph and bone scan may support the diagnosis by allowing for determination of areas of microcalcifications in calciphylaxis. A positive bone scan has a high sensitivity rate of 97% in cases of calciphylaxis. The gold standard for diagnosing calciphylaxis is skin biopsy. Clinicians with adequate training should perform the skin biopsy to prevent further trauma to skin that is already fragile and difficult to heal. Clinicians must weigh the benefits and risks of biopsy; patients with ESRD and classic presentation of calciphylaxis do not require biopsy. The double-punch technique, where a punch biopsy is followed by a second punch inserted through the center of the first punch, can offer an improved yield on the composition of the skin and underlying structures. Once diagnosis of calciphylaxis is made, referral to a dermatologist is helpful for management of the damaged skin.

• #94

  https://link.springer.com/article/10.1007/s12325-020-01504-w

  The characteristic feature of calciphylaxis is the diffuse calcification of small capillaries in adipose tissue. […] In certain cases, medical imaging can be helpful in making a diagnosis. […] The current literature shows that serum laboratory investigations yield little in the way of definitive proof in calciphylaxis cases. […] Cutaneous calciphylaxis can mimic many other skin conditions and diagnosis can be difficult to make, especially in non-uremic patients. Currently, skin biopsy with histological examination is the best method of diagnosing the disease, with medical imaging being helpful in select cases.

• #95 Radiomics-based method for diagnosis of calciphylaxis in patients with chronic kidney disease using computed tomography – Yu – Quantitative Imaging in Medicine and Surgery

  https://qims.amegroups.org/article/view/73274/html

  Calciphylaxis diagnosis is challenging, for which skin biopsy is a gold standard. Nonetheless, it increases the risk of ulceration, bleeding, and necrosis owing to deep incisions. A noninvasive tool for diagnosing calciphylaxis lesions is beneficial for patients with skin lesions and CKD. […] This study aimed to develop radiomic methods using CT as a noninvasive method for calciphylaxis diagnosis. […] In this research, it primarily developed a radiomic method for noninvasive detection of calciphylaxis in patients with CKD. Through this method, calciphylaxis can be detected when invasive procedures are not feasible. […] The model, based on 8 features including 4 first-order features and 4 textural features, showed the highest AUC on the validation data set. […] The exploration of diagnostic models has confirmed the possibility of a non-invasive diagnosis method that might provide clinicians with an alternative when histopathological examination is impossible and thereby reducing the incidence of complications due to invasive operations, especially at the skin lesions.

• #96 A Possible Role For POCUS in the Diagnosis of Calciphylaxis — BROWN EMERGENCY MEDICINE BLOG

  http://brownemblog.com/blogposts/2023/1/16/a-possible-role-for-pocus-in-the-diagnosis-of-calciphylaxis

  Calciphylaxis is a life-threatening vasculopathy that results from the deposition of calcium in the arteriolar vasculature of the deep dermis and subcutaneous adipose tissue, which causes occlusion of the affected blood vessels and overlying tissues and skin. […] Despite the dramatic signs and symptoms associated with calciphylaxis, making this diagnosis solely on clinical grounds can be challenging: retiform purpura is a cutaneous manifestation that commands a wide differential (including cryoglobulinemia, polyarteritis nodosa and other vasculitides, heparin-induced thrombocytopenia, to name a few) and therefore these lesions are not specific to calciphylaxis. […] Recent reports have suggested a possible role for point-of-care ultrasound (POCUS) in the diagnosis of calciphylaxis. […] One report presented a case of penile calciphylaxis that was successfully identified through POCUS after two negative punch biopsies were obtained, suggesting that POCUS may be an additional tool to confirm this condition in the setting of non-diagnostic punch biopsies.

• #97 Radiomics-based method for diagnosis of calciphylaxis in patients with chronic kidney disease using computed tomography – Yu – Quantitative Imaging in Medicine and Surgery

  https://qims.amegroups.org/article/view/73274/html

  Calciphylaxis diagnosis is challenging, for which skin biopsy is a gold standard. Nonetheless, it increases the risk of ulceration, bleeding, and necrosis owing to deep incisions. A noninvasive tool for diagnosing calciphylaxis lesions is beneficial for patients with skin lesions and CKD. […] This study aimed to develop radiomic methods using CT as a noninvasive method for calciphylaxis diagnosis. […] In this research, it primarily developed a radiomic method for noninvasive detection of calciphylaxis in patients with CKD. Through this method, calciphylaxis can be detected when invasive procedures are not feasible. […] The model, based on 8 features including 4 first-order features and 4 textural features, showed the highest AUC on the validation data set. […] The exploration of diagnostic models has confirmed the possibility of a non-invasive diagnosis method that might provide clinicians with an alternative when histopathological examination is impossible and thereby reducing the incidence of complications due to invasive operations, especially at the skin lesions.

• #98 A Possible Role For POCUS in the Diagnosis of Calciphylaxis — BROWN EMERGENCY MEDICINE BLOG

  http://brownemblog.com/blogposts/2023/1/16/a-possible-role-for-pocus-in-the-diagnosis-of-calciphylaxis

  Calciphylaxis is a life-threatening vasculopathy that results from the deposition of calcium in the arteriolar vasculature of the deep dermis and subcutaneous adipose tissue, which causes occlusion of the affected blood vessels and overlying tissues and skin. […] Despite the dramatic signs and symptoms associated with calciphylaxis, making this diagnosis solely on clinical grounds can be challenging: retiform purpura is a cutaneous manifestation that commands a wide differential (including cryoglobulinemia, polyarteritis nodosa and other vasculitides, heparin-induced thrombocytopenia, to name a few) and therefore these lesions are not specific to calciphylaxis. […] Recent reports have suggested a possible role for point-of-care ultrasound (POCUS) in the diagnosis of calciphylaxis. […] One report presented a case of penile calciphylaxis that was successfully identified through POCUS after two negative punch biopsies were obtained, suggesting that POCUS may be an additional tool to confirm this condition in the setting of non-diagnostic punch biopsies.

• #99

  https://journals.lww.com/jfmpc/fulltext/2019/08090/calciphylaxis_and_its_diagnosis__a_review.4.aspx

  Even plain X-rays, nuclear bone scans and circulating fetuin A levels have been reported to aid in the diagnosis, none of these tools have been recommended for clinical use. […] The main requirement in this diagnostic procedure is of a fiber-optic handheld probe that detects the carbonated apatite in calciphylaxis at various sites and depths. […] Best way to confirm calciphylaxis requires biopsy of involved area of skin and the test should be performed whenever the diagnosis is considered. […] Lesional biopsy can demonstrate medial calcification and intimal proliferation of small vessels. Other diagnostic histopathological features are extravascular soft tissue calcification, septal and lobular panniculitis, dermal-epidermal split, and epidermal necrosis. […] The diagnostic criteria based on biopsy sometimes indicate low sensitivity in patients where biopsy specimen lacks calcifications.

• #100 Calciphylaxis and its diagnosis: A review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6820424/

  The primary care physician is the first contact of a patient for the consultation of illness. Early diagnosis and a multi-disciplinary approach are key components of managing this complex disease. […] The disease detection relies on certain set clinical, histopathological and imaging criteria. In patients diagnosed positive for the disease must be taken care because of its increased mortality rate.

• #101 Calciphylaxis, A Case Series: The Importance of Early Detection

  https://scholarlycommons.hcahealthcare.com/hcahealthcarejournal/vol4/iss1/6/

  Calciphylaxis should be suspected in ESRD patients presenting with painful areas of cutaneous induration, and the early recognition of these findings allows for prompt diagnosis and management. […] In all 3 patients, the diagnosis was confirmed histologically, and the management involved the continuation of renal replacement therapy, pain medication, wound debridement, and intravenous (IV) sodium thiosulphate.

• #102 Calciphylaxis: Approach to Diagnosis and Management – Making the Rounds

  https://internalmedicineiowa.org/2020/01/13/calciphylaxis-approach-to-diagnosis-and-management/

  Calciphylaxis is a rare disorder of poor prognosis that can lead to intense, painful lesions involving the skin and subcutaneous tissue. […] The diagnosis of calciphylaxis is complicated by the absence of a gold standard marker of disease such as a clear histopathological finding. […] Late diagnosis and advanced lesions can significantly shorten life expectancy. […] Future research is required to establish clear causal pathways and improve on the treatment options currently available to patients.

• #103 Calciphylaxis in patients with chronic kidney disease: A disease which is still bewildering and potentially fatal | Nefrología

  https://www.revistanefrologia.com/en-calciphylaxis-in-patients-with-chronic-articulo-resumen-S2013251418301068

  Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare syndrome that typically causes skin necrosis and usually affects dialysis patients. […] From a clinical point of view, calciphylaxis may progress from painful purpura to extensive areas of skin necrosis that can potentially lead to superinfection and the death of the patient due to sepsis. […] Calciphylaxis, also called calcific uremic arteriolopathy (CUA), is a clinical syndrome characterized by necrotic ulceration of the skin due to arteriolar calcification of the media plus fibrosis of the intima and subsequent cutaneous ischemia due to thrombosis of the arteriole. […] The diagnosis is mainly clinical, although the role of skin biopsy is very important, especially in doubtful cases. […] The skin biopsy would confirm the diagnosis of calciphylaxis.

• #104 Calciphylaxis or Calcific Uremic Arteriolopathy: Diagnosis not to be Overlooked in Hemodialysis Patients

  https://www.heraldopenaccess.us/openaccess/calciphylaxis-or-calcific-uremic-arteriolopathy-diagnosis-not-to-be-overlooked-in-hemodialysis-patients

  The treatment is less well codified but the most specific treatment used in the first intention is sodium thiosulfate with a dose adapted to renal function from 12.5 to 25mg/day without exceeding 2.5mg/kg which allows dissociation of the salts calcium with antioxidant effect and local vasodilation. […] The life prognosis is poor (60 to 80% of mortality) given the high risk of systemic infection linked to skin lesions. […] The vital prognosis is often engaged.

• #105 Calciphylaxis: Approach to Diagnosis and Management – Making the Rounds

  https://internalmedicineiowa.org/2020/01/13/calciphylaxis-approach-to-diagnosis-and-management/

  Calciphylaxis is a rare disorder of poor prognosis that can lead to intense, painful lesions involving the skin and subcutaneous tissue. […] The diagnosis of calciphylaxis is complicated by the absence of a gold standard marker of disease such as a clear histopathological finding. […] Late diagnosis and advanced lesions can significantly shorten life expectancy. […] Future research is required to establish clear causal pathways and improve on the treatment options currently available to patients.

• #106 Calciphylaxis, A Case Series: The Importance of Early Detection

  https://scholarlycommons.hcahealthcare.com/hcahealthcarejournal/vol4/iss1/6/

  Calciphylaxis should be suspected in ESRD patients presenting with painful areas of cutaneous induration, and the early recognition of these findings allows for prompt diagnosis and management. […] In all 3 patients, the diagnosis was confirmed histologically, and the management involved the continuation of renal replacement therapy, pain medication, wound debridement, and intravenous (IV) sodium thiosulphate.

• #107 What Is Your Diagnosis? Calciphylaxis | MDedge

  https://community.the-hospitalist.org/content/what-your-diagnosis-calciphylaxis

  On biopsy, characteristic findings established the diagnosis of calciphylaxis. Furthermore, the pattern of ulceration in pressure areas associated with the patient lift indicated that infection was not the precipitating event. […] Treatment of calciphylaxis must be individualized. It is important to treat any underlying disease and remove any precipitating factors. In the case of severe renal disease, a multidisciplinary approach typically is necessary. Correction of serum calcium and phosphate levels, rapid and safe rehydration, dialysis, and intravenous bisphosphonates all may be needed in patients with chronic kidney disease. Surgical management of the nonhealing necrotic skin lesions via surgical debridement and skin grafting has been reported to improve overall morbidity and survival rates.

• #108 Calciphylaxis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/calciphylaxis/symptoms-causes/syc-20370559

  Calciphylaxis treatments include various medicines, procedures and surgery. Treatment can help prevent blood clots and infections, reduce calcium buildups, heal sores, and ease pain. […] The exact cause of calciphylaxis isn’t known. The disease involves the buildup of calcium in the smallest parts of the arteries in fat tissues and skin. […] Finding and treating any infections early is key to preventing serious complications. […] There isn’t a clear way to prevent calciphylaxis. But if you are on dialysis or have low kidney function due to advanced chronic kidney disease, it’s important to keep blood levels of calcium and phosphorus under control.

• #109 What Is Your Diagnosis? Calciphylaxis | MDedge

  https://community.the-hospitalist.org/content/what-your-diagnosis-calciphylaxis

  On biopsy, characteristic findings established the diagnosis of calciphylaxis. Furthermore, the pattern of ulceration in pressure areas associated with the patient lift indicated that infection was not the precipitating event. […] Treatment of calciphylaxis must be individualized. It is important to treat any underlying disease and remove any precipitating factors. In the case of severe renal disease, a multidisciplinary approach typically is necessary. Correction of serum calcium and phosphate levels, rapid and safe rehydration, dialysis, and intravenous bisphosphonates all may be needed in patients with chronic kidney disease. Surgical management of the nonhealing necrotic skin lesions via surgical debridement and skin grafting has been reported to improve overall morbidity and survival rates.

• #110 What Is Your Diagnosis? Calciphylaxis | MDedge

  https://community.the-hospitalist.org/content/what-your-diagnosis-calciphylaxis

  On biopsy, characteristic findings established the diagnosis of calciphylaxis. Furthermore, the pattern of ulceration in pressure areas associated with the patient lift indicated that infection was not the precipitating event. […] Treatment of calciphylaxis must be individualized. It is important to treat any underlying disease and remove any precipitating factors. In the case of severe renal disease, a multidisciplinary approach typically is necessary. Correction of serum calcium and phosphate levels, rapid and safe rehydration, dialysis, and intravenous bisphosphonates all may be needed in patients with chronic kidney disease. Surgical management of the nonhealing necrotic skin lesions via surgical debridement and skin grafting has been reported to improve overall morbidity and survival rates.

• #111 Calciphylaxis – Diagnosis and Management | Vascular Calcification

  https://woundeducators.com/calciphylaxis-diagnosis-and-management/?srsltid=AfmBOoqvfZrFdge3xAbmgba-XsDz3_AViaS5LlzqaHXUZ6OtO4eShxAB

  If calciphylaxis is suspected, the diagnosis should be confirmed histologically in order to differentiate the condition from other similar vasculopathies which may be presented by the same patient types. […] Once diagnosis has been confirmed, effective wound care becomes the cornerstone of management, and should be based around periodic debridement of necrotic tissue and proven wound management strategies. […] The lack of evidence for the benefit of any treatment for calciphylaxis is disappointing, and contributes to the overall dismal prognosis for this condition.

• #112 Calciphylaxis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK519020/

  Sodium thiosulfate is now routinely used off-label in the treatment of calciphylaxis. In a case series evaluating the efficacy of sodium thiosulfate, more than 70% of patients receiving it had improvement or resolution of their skin lesions, and mortality rate was lower than historically published data. […] A definitive diagnosis for calciphylaxis is made with a skin biopsy which predominantly shows arteriolar medial layer calcification, although the diagnosis can at times be made on clinical grounds.

• #113 What Is Your Diagnosis? Calciphylaxis | MDedge

  https://community.the-hospitalist.org/content/what-your-diagnosis-calciphylaxis

  Currently, there is no standard therapy in place for treatment of calciphylaxis but several are under investigation. Most notably, sodium thiosulfate, an inorganic salt that promotes dissolution of calcium deposits by chelating calcium, has shown rapid improvement when other treatments have failed. […] Calciphylaxis is a rare condition with a high mortality rate, most often due to septicemia from secondary infection of skin lesions. The exact pathogenic mechanisms are vague but may be the result of several precipitating factors. Local wound care and therapeutics should be used synergistically and individualized for each patient with calciphylaxis.

• #114 Perioperative Management of Calciphylaxis: Literature Review and Treatment Recommendations | Published in Orthopedic Reviews

  https://orthopedicreviews.openmedicalpublishing.org/article/37573-perioperative-management-of-calciphylaxis-literature-review-and-treatment-recommendations

  There are also no comprehensive guidelines for perioperative management of patients with calciphylaxis. […] A retrospective analysis of cases of 5 patients treated with parathyroidectomy reported no specific additional risks of surgery. […] Pain control should be aggressively pursued using a multimodal approach. […] For this study, we identified 3 areas of interest that should be considered in postoperative care. […] Given the high rates of morbidity and mortality associated with calciphylaxis, discussing palliative care options with patients early in the treatment process may be useful to maximize quality of life.

• #115 Calciphylaxis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/calciphylaxis/symptoms-causes/syc-20370559

  Calciphylaxis treatments include various medicines, procedures and surgery. Treatment can help prevent blood clots and infections, reduce calcium buildups, heal sores, and ease pain. […] The exact cause of calciphylaxis isn’t known. The disease involves the buildup of calcium in the smallest parts of the arteries in fat tissues and skin. […] Finding and treating any infections early is key to preventing serious complications. […] There isn’t a clear way to prevent calciphylaxis. But if you are on dialysis or have low kidney function due to advanced chronic kidney disease, it’s important to keep blood levels of calcium and phosphorus under control.

• #116 Perioperative Management of Calciphylaxis: Literature Review and Treatment Recommendations | Published in Orthopedic Reviews

  https://orthopedicreviews.openmedicalpublishing.org/article/37573-perioperative-management-of-calciphylaxis-literature-review-and-treatment-recommendations

  Calciphylaxis is a serious and rare medical condition that leads to substantial clinical manifestations including pain, creating perioperative and treatment challenges. […] Currently, no standard treatment protocol exists for calciphylaxis nor are comprehensive guidelines available for perioperative care of patients with calciphylaxis. […] The resultant clinical effects of calciphylaxis create perioperative treatment challenges. […] The purpose of this review was to evaluate existing literature in order to offer guidance on treating patients with this challenging disease through the perioperative period. […] Although a standard protocol does not exist for treating calciphylaxis, the literature search identified several common therapies being used including intravenous sodium thiosulfate, parathyroidectomy, surgical debridement, bisophosphonate, cinacalcet, and hyperbaric oxygen therapy.

• #117 Perioperative Management of Calciphylaxis: Literature Review and Treatment Recommendations | Published in Orthopedic Reviews

  https://orthopedicreviews.openmedicalpublishing.org/article/37573-perioperative-management-of-calciphylaxis-literature-review-and-treatment-recommendations

  There are also no comprehensive guidelines for perioperative management of patients with calciphylaxis. […] A retrospective analysis of cases of 5 patients treated with parathyroidectomy reported no specific additional risks of surgery. […] Pain control should be aggressively pursued using a multimodal approach. […] For this study, we identified 3 areas of interest that should be considered in postoperative care. […] Given the high rates of morbidity and mortality associated with calciphylaxis, discussing palliative care options with patients early in the treatment process may be useful to maximize quality of life.

• #118 Perioperative Management of Calciphylaxis: Literature Review and Treatment Recommendations | Published in Orthopedic Reviews

  https://orthopedicreviews.openmedicalpublishing.org/article/37573-perioperative-management-of-calciphylaxis-literature-review-and-treatment-recommendations

  There are also no comprehensive guidelines for perioperative management of patients with calciphylaxis. […] A retrospective analysis of cases of 5 patients treated with parathyroidectomy reported no specific additional risks of surgery. […] Pain control should be aggressively pursued using a multimodal approach. […] For this study, we identified 3 areas of interest that should be considered in postoperative care. […] Given the high rates of morbidity and mortality associated with calciphylaxis, discussing palliative care options with patients early in the treatment process may be useful to maximize quality of life.

• #119

  https://scholars.duke.edu/publication/1590446

  Calciphylaxis is an uncommon but devastating disorder characterized by vascular calcification and subsequent cutaneous tissue necrosis. This results in exquisitely painful and slow healing wounds that portend exceptionally high morbidity and mortality. The diagnosis of this condition can be complicated because there are no conclusive serologic, radiographic or visual signs that this disease is manifesting. The differential of tissue necrosis is broad, and identifying calciphylaxis requires an adroit understanding of the risk factors and physical signs that should raise suspicion of this condition. […] Reviews on this subject are uncommon and lack directed commentary from disease experts on the best diagnostic approach for patients suffering from this disease. The goal of this article is to update practicing dermatologists on the current standard of care for calciphylaxis.

• #120 Calciphylaxis and its diagnosis: A review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6820424/

  The primary care physician is the first contact of a patient for the consultation of illness. Early diagnosis and a multi-disciplinary approach are key components of managing this complex disease. […] The disease detection relies on certain set clinical, histopathological and imaging criteria. In patients diagnosed positive for the disease must be taken care because of its increased mortality rate.

• #121 Calciphylaxis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK519020/

  Calciphylaxis typically presents with extremely painful ischemic cutaneous lesions or painful subcutaneous nodules without skin changes, although at times, pain may precede the development of the lesions. […] Diagnosing calciphylaxis requires a high index of suspicion. A definitive diagnosis is made after a skin biopsy of one of the lesions. The histologic evaluation shows medial calcification of dermal arterioles or small arteries and may show fibrointimal hyperplasia, microthrombi, and vascular narrowing or occlusion, often with evidence of necrosis. […] The need for biopsy in the diagnosis of calciphylaxis is debatable. While a biopsy is often performed to differentiate early stages of calciphylaxis from other skin lesions, the biopsy itself carries some risk, particularly for patients with only subcutaneous nodules.

• #122 Calciphylaxis Diagnosis and Management in Primary Care

  https://www.clinicaladvisor.com/features/calciphylaxis-diagnosis-treatment-primary-care/

  Calciphylaxis should be considered in patients presenting with painful nonhealing skin ulcers who have chronic kidney disease, obesity, have been on hemodialysis, and/or are of White race and female sex. Skin changes associated with calciphylaxis have sudden onset and progress rapidly to open ulcerations and then necrosis. A thorough physical examination of the patient, preferably with the torso and lower extremities exposed, can assist the clinician in visualizing the patients lesions and assessing skin integrity. A painful necrotic ulcer covered with black eschar that started as painful ecchymoses is usually the presenting symptom; patients often mistake initial reddening for a bruise and delay seeking medical care. The presence of malodorous, dusky, and/or necrotic lesions should increase suspicion of calciphylaxis. Clinical findings include purple or red net-like areas of ecchymoses that are painful or nonhealing ulcers that are painful. Imaging studies such as radiograph and bone scan may support the diagnosis by allowing for determination of areas of microcalcifications in calciphylaxis. A positive bone scan has a high sensitivity rate of 97% in cases of calciphylaxis. Laboratory tests such as renal function tests, serum calcium, alkaline phosphatase, phosphorous, and vitamin D can evaluate potential risk factors. The gold standard for diagnosing calciphylaxis is skin biopsy. Clinicians with adequate training should perform the skin biopsy to prevent further trauma to skin that is already fragile and difficult to heal. Clinicians must weigh the benefits and risks of biopsy; patients with ESRD and classic presentation of calciphylaxis do not require biopsy. The double-punch technique, where a punch biopsy is followed by a second punch inserted through the center of the first punch, can offer an improved yield on the composition of the skin and underlying structures. Once diagnosis of calciphylaxis is made, referral to a dermatologist is helpful for management of the damaged skin.

• #123

  https://link.springer.com/article/10.1007/s12325-020-01504-w

  The characteristic feature of calciphylaxis is the diffuse calcification of small capillaries in adipose tissue. […] In certain cases, medical imaging can be helpful in making a diagnosis. […] The current literature shows that serum laboratory investigations yield little in the way of definitive proof in calciphylaxis cases. […] Cutaneous calciphylaxis can mimic many other skin conditions and diagnosis can be difficult to make, especially in non-uremic patients. Currently, skin biopsy with histological examination is the best method of diagnosing the disease, with medical imaging being helpful in select cases.

• #124 Advanced-stage calciphylaxis: Think before you punch | Cleveland Clinic Journal of Medicine

  https://www.ccjm.org/content/83/8/562

  In the advanced stages, the diagnosis of calciphylaxis is clinically more evident, and the differential diagnosis usually narrows. […] Well-demarcated, necrotic, indurated lesions that are bilateral in a patient with end-stage renal disease without shock makes the diagnosis very likely. […] Biopsy for histologic confirmation of calciphylaxis can increase the risk of infection and sepsis. […] Since extensive necrosis predisposes to a negative biopsy, a high clinical suspicion should drive early treatment of calciphylaxis. […] Noninvasive imaging studies such as plain radiography and bone scintigraphy can aid the diagnosis by detecting moderate to severe soft-tissue vascular calcification in these areas.

• #125 Calciphylaxis and its diagnosis: A review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6820424/

  The primary care physician is the first contact of a patient for the consultation of illness. Early diagnosis and a multi-disciplinary approach are key components of managing this complex disease. […] The disease detection relies on certain set clinical, histopathological and imaging criteria. In patients diagnosed positive for the disease must be taken care because of its increased mortality rate.

• #126 Calciphylaxis: Pictures, Definition, Symptoms, Treatment, and Outlook

  https://www.healthline.com/health/calciphylaxis

  Successful management of calciphylaxis symptoms is possible, and early diagnosis and treatment can lead to better outcomes even without a cure. The survival rate is expected to improve as more research is performed. […] Calciphylaxis often occurs in people with end stage kidney disease and those who are on dialysis. But it can sometimes occur in people with regularly functioning kidneys. Early diagnosis may improve the outcome.

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